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Ana Sayfa : Recent BD News : Review of published papers : Clinical : Listed during April 2005 – October 2006

Listed during April 2005 – October 2006

Listed during April 2005 – October 2006
Prepared by: M. Schirmer (Austria) – K. Calamia (USA)

We would like to resume our listing of the clinical literature on Behcet’s disease (BD) which have appeared in Pub Med between April and October of 2006 for all members of the ISBD.
Indeed, we found an overwhelming number of original publications and 106 case reports in PubMed published between April 2005 and October 2006. Although a direct comparison to publication trends in other diseases has not been performed it indicates that there is a continued interest for BD in the international community and BD patients often present with diagnostic and therapeutic challenges interesting for the readers of international journals.
Most importantly the publications originate from multiple medical specialties, reflecting the systemic nature of BD. Gastroenterological studies for example showed that capsule endoscopy is a useful diagnostic tool for diagnosis of obscure gastrointestinal bleeding. This technique has been useful in detecting lesions in BD patients, in patients with chronic diarrhoa and in estimating the extension of Crohn’s disease. Another report from Korea suggested that gastrointestinal surgery should be strongly considered in BD when the steroid dose exceeds 40mg/day.
This time the publications are summarized in more subspecialties than in the previous reviews, hopefully making an update on current literature more convenient. Please let us know if you have any suggestions for this effort.
M. Schirmer (Elisabethinen-Hospital Klagenfurt, Austria) 
K.T. Calamia (MAYO Clinic Jacksonville, FL)

OPHTHMOLOGY (except studies focusing on TNF-blocking agents)
CARDIOVASCULAR DISEASE (including surgical treatment)
MORE PAPERS (including interventional trials) ...

  1. Tumour necrosis factor alpha inhibitors in the treatment of childhood uveitis. Saurenmann RK, Levin AV, Rose JB, Parker S, Rabinovitch T, Tyrrell PN, Feldman BM, Laxer RM, Schneider R, Silverman ED. Rheumatology (Oxford). 2006 Aug;45(8):982-9. Epub 2006
    OBJECTIVE: To describe the efficacy of anti-TNF-alpha agents in the treatment of childhood uveitis. METHODS: We performed a retrospective chart review of all children with uveitis treated with TNF-alpha blockers at The Hospital for Sick Children, Toronto. RESULTS: Twenty-one children with uveitis were treated with the anti-TNF-alpha agents etanercept (11 patients) and infliximab (13 patients), resulting in 24 treatment courses. All patients had persistently active uveitis despite treatment with at least one standard immunosuppressive drug before the start of anti-TNF-alpha therapy. Six of 21 patients (29%) had idiopathic uveitis. In the other 15 patients, the underlying disease was juvenile idiopathic arthritis in 12 (57%), Behcet disease in two (9%) and sarcoidosis in one (5%). Response to etanercept treatment was good in 27%, moderate in 27% and poor in 45% of patients. Response to infliximab treatment was good in 38%, moderate in 54% and poor in 8% of patients. The difference in the percentage of patients with a moderate or good response was statistically significant (P = 0.0481). We also observed a lower rate of complications, such as new-onset or worsening glaucoma or cataract in the infliximab-treated group. CONCLUSION: Anti-TNF-alpha treatment was beneficial in a high percentage of patients with childhood uveitis refractory to standard immunosuppressive treatment. Infliximab resulted in better clinical responses with less ocular complications than etanercept.

  1. The clinical application of etanercept in Chinese patients with rheumatic diseases. Chou CT. Mod Rheumatol. 2006;16(4):206-13.
    Over a 2-year period, to evaluate the efficacy and safety of biologic agents, etanercept (25 mg twice per week, s.c.) was used to treat 57 rheumatoid arthritis (RA) patients, 9 ankylosing spondylitis (AS) patients, 6 psoriatic arthritis (PSA) patients, and 4 juvenile rheumatoid arthritis (JRA) patients. In addition to inflammatory arthritis, I have used this tumor necrosis factor (TNF) blocker in other rheumatic diseases including idiopathic thrombocytopenic purpura (ITP), Behcet's disease with intractable oral ulcer, fibromyalgia syndrome, and systemic lupus erythematosis with intractable pleural effusion and acute lumbar disc herniation. For RA, after 6 months of etanercept treatment, all the parameters including number of swollen joints, number of tender joints, disease activity score, erythrocyte sedimentation rate, C-reactive protein, and global health status were rapidly improved (P < 0.001 or P < 0.0001). The anticyclic citrullinated peptide (anti-CCP) antibody and rheumatoid factor also significantly declined. For spondyloarthropathy, it also gave a similar effect as to RA. Both Bath Ankylosing Spondylitis Disease Activity Index and Bath Ankylosing Spondylitis Functional Index also improved. One of the two cases with Behcet's disease with intractable oral ulcer had a long-term remission after etanercept. The other Behcet's disease patient with oral ulcer and another with ITP obtained a good response temporarily. The short-term use of etanercept (<3 months) did not bring a significant effect for cases of fibromyalgia syndrome, pleural effusion, and lumbar disc herniation. In conclusion, a dramatic and rapid clinical response in different kinds of arthritis patients can be achieved by etanercept. Moreover, the TNF-alpha inhibitor also can treat other severe rheumatic-related symptoms. In general, except for a few cases with infection and two cases with malignancy, etanercept was safe in our arthritis patients. We need to study a larger number of patients in order to better understand the efficacy and safety of etanercept.

  1. Efficacy of infliximab in the treatment of uveitis that is resistant to treatment with the combination of azathioprine, cyclosporine, and corticosteroids in Behcet's disease: an open-label trial. Tugal-Tutkun I, Mudun A, Urgancioglu M, Kamali S, Kasapoglu E, Inanc M, Gul A. Arthritis Rheum. 2005 Aug;52(8):2478-84.
    OBJECTIVE: To investigate the effect of infliximab on the frequency of uveitis attacks and the visual prognosis in male patients with Behcet's disease in whom uveitis was resistant to combination therapy with corticosteroids, azathioprine, and cyclosporine. METHODS: The study group comprised patients who were receiving combination therapy but experienced at least 2 attacks of posterior uveitis/panuveitis or retinal vasculitis during the 6-month period prior to enrollment. Infliximab infusions (5 mg/kg) were administered at weeks 0, 2, 6, and 14. Weeks 0-22 were defined as the infusion period, and weeks 23-54 were defined as the observation period. Patients continued to receive azathioprine and corticosteroids, but cyclosporine was discontinued after the screening visit. The primary outcome measures were the absence of uveitis attacks during the infusion period (remission), and the absence of uveitis attacks throughout the study period (sustained remission). RESULTS: Thirteen patients were enrolled in the study. Thirty-two uveitis attacks involving the posterior segment occurred during the previous-treatment period. During the infusion period, 4 patients (30.8%) remained attack-free, and 9 patients had a total of 13 uveitis attacks. Ten of these attacks (76.9%) occurred at either week 14 or week 22. One of 13 patients fulfilled the definition of sustained remission, and the remaining 12 patients had a total of 36 uveitis attacks during the observation period. The mean number of uveitis attacks and daily corticosteroid doses were significantly lower during the infusion period than during the previous-treatment period or the observation period. Although potential visual acuity was regained following infliximab infusion, this beneficial effect was not preserved until week 54. None of the patients experienced a serious adverse event. CONCLUSION: The results of this trial suggest that infliximab is effective in suppressing the occurrence of uveitis attacks, has a corticosteroid-sparing effect, and has favorable implications for the visual prognosis of patients with resistant Behcet's uveitis.

  1. Long-term treatment of refractory posterior uveitis with anti-TNFalpha (infliximab). Benitez-del-Castillo JM, Martinez-de-la-Casa JM, Pato-Cour E, Mendez-Fernandez R, Lopez-Abad C, Matilla M, Garcia-Sanchez J. Eye. 2005 Aug;19(8):841-5.
    PURPOSE: To evaluate the long-term efficacy and safety of infliximab as treatment for noninfectious posterior uveitis. METHODS: An open-label clinical trial including seven patients (12 eyes) with posterior uveitis refractory to conventional treatment regimens with corticosteroids and at least one immunosuppressive agent. Three intravenous doses of 5 mg/kg of infliximab were administered at weeks 0, 2, and 6. Infliximab infusion was repeated in patients undergoing a relapse of uveitis after initial remission. Improvement was defined as amelioration of visual acuity or disappearance of retinal exudates and/or haemorrhages, decreased macular oedema and/or vitreous opacities. All patients were followed up for at least 36 months. RESULTS: Six of the seven patients (five diagnosed with Behcet's disease and one diagnosed with sarcoidosis) showed a significant improvement after the first infliximab dose. Only in one patient diagnosed with chronic idiopathic multifocal choroiditis did the drug have no effect, and this patient was withdrawn from the study. At the end of follow-up, one eye had lost one line of vision and three eyes showed improved vision. All eyes had improved in terms of signs of inflammation. No adverse effects of treatment were observed. CONCLUSION: Infliximab is efficient and safe for the long-term management of refractory posterior uveitis, especially in patients with predominant retinal vasculitis and vitritis.

  1. Anti-TNF-alpha therapy for sight threatening uveitis. Lindstedt EW, Baarsma GS, Kuijpers RW, van Hagen PM. Br J Ophthalmol. 2005 May;89(5):533-6.
    AIM: To describe the effect of additional treatment with anti-TNF-alpha therapy in a case series of 13 patients with serious sight threatening uveitis. METHODS: 13 patients with serious sight threatening uveitis were included, of whom six had Behcet's disease, five had idiopathic posterior uveitis, one had sarcoidosis, and one birdshot retinochoroiditis. Onset and course of ocular inflammation, inflammatory signs, and visual acuity were assessed. Patients were treated with 200 mg (approximately 3 mg/kg) infliximab infusion. Repeat infusions were given based on clinical response. RESULTS: Infliximab treatment resulted in an effective suppression of ocular inflammation in all patients. In patients with non-Behcet's disease uveitis visual acuity in six out of eight improved or was stable. In patients with Behcet's disease visual acuity in five out of six improved or was stable. CONCLUSION: Anti-TNF-alpha treatment may be of value in the treatment of uveitis, and in patients with Behcet's disease, leading to suppression of ocular inflammation, vasculitis, and improvement of vision in the majority. Based on these results a controlled masked study is warranted.

OPHTHMOLOGY (except studies focusing on TNF-blocking agents)
  1. Repeated intravitreal triamcinolone injections in Behcet disease that is resistant to conventional therapy: one-year results. Ohguro N, Yamanaka E, Otori Y, Saishin Y, Tano Y. Am J Ophthalmol. 2006 Jan;141(1):218-20.
    PURPOSE: To determine whether repeated intravitreal triamcinolone (IVTA) injections in patients with Behcet disease that is resistant to conventional therapy will prevent recurrences of uveitis. DESIGN: Prospective preliminary clinical trial. METHODS: Nine eyes of five patients with Behcet disease with sight-threatening uveitis that was resistant to conventional therapy received repeated IVTA injections. Each patient was examined monthly, and IVTA was given when the TA particles that remained in the vitreous were judged to be gone before the next visit. RESULTS: Repeated IVTA completely prevented the recurrence of uveitis in eight eyes, and the one eye that had an attack experienced the uveitis when the TA particles disappeared quicker than expected. It was possible to stop systemic corticosteroid and/or immunosuppressive agents within 1 year after IVTA in three patients. CONCLUSION: These results indicate that repeated IVTA will prevent the recurrence of uveitis in patients with Behcet disease with frequent ocular attacks that is resistant to conventional therapy.

  1. Pars plana vitrectomy for ocular complications of Behcet's disease. Sullu Y, Alotaiby H, Beden U, Erkan D. Ophthalmic Surg Lasers Imaging. 2005 Jul-Aug;36(4):292-7.
    BACKGROUND AND OBJECTIVES: To evaluate the outcomes of vitreoretinal surgery for the ocular complications of Behcet's disease. PATIENTS AND METHODS: This retrospective study included 20 eyes of 15 patients with Behcet's disease (9 men, 6 women; mean age at surgery, 31.0 +/- 1.8 years; range, 21-44 years) who had undergone vitreoretinal surgery for Behcet's disease at Ondokuz Mayis University, Faculty of Medicine, Department of Ophthalmology, Samsun, Turkey, between January 2000 and September 2003. Postoperative visual acuity outcome, course of uveitis, and complications were evaluated in all patients. RESULTS: Indications for surgery were vitreous opacities in 10 eyes (50%), cystoid macular edema in 5 eyes (25%), epiretinal membrane in 3 eyes (15%), and vitreous hemorrhage in 2 eyes (10%). At the last postoperative follow-up, visual acuity had increased 2 Snellen lines or more in 10 (50%) of 20 eyes. Cystoid macular edema completely improved in 3 (60%) of 5 eyes after vitrectomy. Uveitis attacks significantly decreased during follow-up (P = .015). Immunosuppressive treatment was stopped in 11 (73%) of 15 patients. Retinal detachment and phthisis were not observed in any eye. CONCLUSION: Vitreoretinal surgery may be useful in the treatment of posterior segment complications of Behcet's disease.

  1. Vitrectomy for persistent panuveitis in Behcet's disease. Ahn JK, Chung H, Yu HG. Ocul Immunol Inflamm. 2005 Dec;13(6):447-53.
    PURPOSE: To determine whether vitrectomy is safe and effective for the management of vitreoretinal complications in Behcet's patients with persistent panuveitis. METHODS: Patients who had undergone therapeutic vitrectomy for Behcet's panuveitis were reviewed retrospectively. Those patients who had shown persistent uveitis despite maximum medical therapy were included. Surgical outcomes were measured by an improvement in vision of 0.3 logarithm of minimum angle of resolution, acute relapse rates, and postoperative complications. RESULTS: Twenty-one patients with a mean postoperative follow-up of 30 months were identified. For almost all patients visual acuity and inflammatory control were significantly improved after vitrectomy. However, patients with optic disc neovascularization (NVD) showed no improvement in their vision or inflammatory control. The postoperative rise of intraocular pressure was frequently associated with intravitreal triamcinolone acetonide injections. CONCLUSIONS: Our study suggests that vitrectomy may be both safe and effective for the management of vitreoretinal complications in Behcet's patients with persistent uveitis. Patients with NVD, however, may have poor outcomes.

  1. Neovascularization of the optic disc in Behcet's disease. Tugal-Tutkun I, Onal S, Altan-Yaycioglu R, Kir N, Urgancioglu M. Jpn J Ophthalmol. 2006 May-Jun;50(3):256-65.
    PURPOSE: To investigate the mechanisms involved in the development of neovascularization of the optic disc (NVD) in Behcet's disease and to evaluate the effects of medical and laser treatments. METHODS: This is a retrospective study of 26 consecutive Behcet's patients (38 eyes) with NVD who presented between 1990 and 2004 at our university hospital. Information on age at presentation, sex, disease duration, laterality of NVD, ocular findings, fluorescein angiographic findings, visual acuity, medical treatment, laser photocoagulation, surgical procedures, and the follow-up period was collected. Eyes with diffuse capillary leakage on fluorescein angiography were defined as having inflammation-induced NVD, and eyes with extensive retinal capillary nonperfusion were defined as having ischemia-induced NVD. RESULTS: Eighteen patients were male, and eight were female. Mean age at presentation was 25.4 +/- 4.9 years. Median disease duration was 5.5 months. Median follow-up was 24 months. Twelve patients had bilateral NVD; 14 had unilateral NVD. Inflammation-induced NVD was seen in 87% of the eyes, and ischemia-induced NVD in 13%. Initial treatment with high-dose corticosteroids combined with conventional immunosuppressive agents was effective in 45% of the eyes with inflammation-induced NVD. Retinal laser photocoagulation was effective in three of five eyes with ischemia-induced NVD. Treatment with interferon alpha-2a resulted in resolution of NVD in all seven patients who received this agent for inflammation- or ischemia-induced persistent NVD. Final visual acuity was less than 0.1 in 24% of the eyes. CONCLUSIONS: Inflammatory mechanisms seem to predominate in the pathogenesis of NVD in Behcet's disease. The results of this study suggest that the response to intensive anti-inflammatory and conventional immunosuppressive treatment is not satisfactory; retinal laser photocoagulation may be ineffective even in eyes with retinal ischemia, but interferon alpha-2a seems to be effective for the treatment of Behcet's patients with NVD.

  1. Granulocytapheresis in patients with refractory ocular Behcet's disease. Namba K, Sonoda KH, Kitamei H, Shiratori K, Ariyama A, Iwabuchi K, Onoe K, Saniabadi AR, Inaba S, Ishibashi T, Ohno S. J Clin Apher. 2006 Jul;21(2):121-8.
    Intraocular inflammation (uveoretinitis) is one major complication of Behcet's disease (BD) and responds poorly to drug therapy. This open prospective study was to assess the efficacy of selective granulocytapheresis in patients with refractory uveoretinitis of BD. Fourteen patients aged 20-56 years were treated. Granulocytapheresis was done with an Adacolumn filled with cellulose acetate leucocyte carries or beads that adsorb granulocytes and monocytes from the blood in the column. Each patient received 5 Adacolumn sessions at one session/week over 5 consecutive weeks. The study was designed to allow each patient to serve as his or her own control. The total numbers of ocular attacks (OA) were monitored for 6 months before and after 5 Adacolumn sessions. The number of OA (mean +/- SD) per patient for the 6 months before Adacolumn was 4.21 +/- 1.6 and for the 6 months post Adacolumn was 2.93 +/- 1.39 ( P = 0.0275). Nine patients (64%) improved and 5 did not change or worsened. Further, for a sub-group (n = 7) with duration of BD > or =5 years, the number of OA were 4.71 +/- 1.89 for the first 6 months and 2.29 +/- 1.38 for the second 6 months ( P = 0.0054). The corresponding values for a sub-group (n = 7) with duration of BD<5 years were 3.71 +/- 1.25 and 3.57 +/- 1.13, indicating that patients with long duration of BD are better responders. We conclude that granulocytapheresis might be effective and safe for patients with refractory ocular BD. Further studies are necessary to fully evaluate the clinical efficacy of granulocytapheresis for BD.

  1. Doppler ultrasonography findings in ocular Behcet's disease. Yanik B, Conkbayir I, Berker N, Songgur M, Keyik B, Kursun N, Hekimoglu B. Clin Imaging. 2006 Sep-Oct;30(5):303-8.
    The aim of this prospective study was to determine and compare the orbital Doppler ultrasonography parameters of patients with Behcet's disease (with or without ocular involvement) with those of healthy subjects. We evaluated ophthalmic artery (OA), central retinal artery, posterior ciliary artery (PCA), central retinal vein, and superior ophthalmic vein (SOV) flow velocities and resistance indices (RIs). Detection of the decreased flow velocities in the OA and SOV and the increased RI in the OA and PCA might allow the identification of active period of patients with Behcet's disease.

  1. Color Doppler imaging of ocular hemodynamic changes in Behcet disease and uveitis patients with different etiologies. Oner A, Akal A, Erdogan N, Dogan H, Oner M. Curr Eye Res. 2006 Jun;31(6):519-23.
    PURPOSE: To investigate blood flow velocity changes and resistivity index values of orbital vessels in Behcet disease with or without ocular involvement, in uveitis patients with different etiologies, and in healthy volunteers. METHODS: Subjects were divided into four groups: those with ocular involvement in Behcet disease (group I), those without ocular involvement in Behcet disease (group II), uveitis patients with different etiologies (group III), and a control group (group IV). Twenty-seven eyes in group I, 14 eyes in group II, 25 eyes in group III, and 27 eyes in group IV were investigated. The blood flow in the central retinal artery (CRA), ophthalmic artery (OA), nasal posterior ciliary artery (NPCA), and the temporal posterior ciliary artery (TPCA) was measured using color Doppler ultrasonography (CDU) to determine the peak systolic velocity (PSV), end diastolic velocity (EDV), and resistivity index (RI). RESULTS: The PSV and EDV value of CRA in groups I, II, and III were found to be significantly lower than those in the control group (p < 0.05 for each). The PSV and EDV values of TPCA and NPCA in groups I, II, and III were significantly lower than those in the control group (p < 0.05 for each). Resistivity indexes of all arteries were higher in groups I, II, and III than the control group. The PSV and EDV in CRA, TPCA, and NPCA in Behcet disease patients with ocular involvement were significantly lower than all other groups (p < 0.05). CONCLUSIONS: There are significant reductions in the blood flow values of the orbital arteries in patients with Behcet disease and uveitis patients with different etiologies when compared with healthy volunteers. The decrease in blood flow values in Behcet disease is more evident in patients with ocular involvement than the patients without ocular involvement. This might be the result of occlusive vasculitis, which is frequently seen in the retinal vessels of patients with Behcet disease.

  1. Visual evoked potential findings in Behcet's disease without neurological manifestations. Anlar O, Akdeniz N, Tombul T, Calka O, Bilgili SG. Int J Neurosci. 2006 Mar;116(3):281-7.
    Behcet's disease (BD) is a chronic, recurrent multisystem inflammatory disorder firstly described by Turkish dermatologist Dr. Hulusi Behcet in 1937. The classic triad consists of recurrent oral and genital ulcerations and uveitis. The article presents the value of visual evoked potential findings of a series of 44 patients with BD without neurological manifestations seen at the Medical Hospital in Neurology and Dermatology clinics over the past 8 years. The mean latency value of positive peak P100 in BD patients was significantly delayed compared to that of control subjects (patients's mean: 105.6 ms in right eye and 107.7 ms in left eye; control subject's mean: 101.4 ms in right eye and 101.7 ms in left eye).

  1. Risk and prognostic factors of poor visual outcome in Behcet's disease with ocular involvement. Takeuchi M, Hokama H, Tsukahara R, Kezuka T, Goto H, Sakai J, Usui M. Graefes Arch Clin Exp Ophthalmol. 2005 Nov;243(11):1147-52. Epub 2005 Jun 28.
    PURPOSE: The purpose of the study was to determine factors correlated with the progression of irreversible visual disturbance in Behcet's disease (BD) with ocular involvement. METHODS: Forty-seven BD patients with ocular inflammation, who presented with the first ocular episode, and who had been followed continuously for 5-10 years in our hospital, were studied. Charts were reviewed for gender, onset age of uveitis, complete or incomplete type BD, HLA-B51 status, final visual acuity at the last remission period, mean number of ocular attacks per year, and clinical findings of iridocyclitis with profuse hypopyon, strong vitreous opacity blocking the observation of retinal vessels, diffuse retinal vasculitis, and exudates with hemorrhage within the retinal vascular arcade. RESULTS: Patients with a visual acuity of < or =20/200 and those with >20/200 differed significantly in the mean number of ocular attacks per year and clinical findings of strong vitreous opacity and exudates within the retinal vascular arcade, but not with regard to the other factors. In addition, the frequency of ocular attacks showed a significant negative correlation with the outcome of visual acuity. Logistic regression analysis indicated a significant association of an average of more than three ocular attacks per year, strong vitreous opacity, and exudates within the retinal vascular arcade with poor visual outcome. CONCLUSIONS: This study indicates that more than three ocular attacks per year, strong vitreous opacity, and exudates within the retinal vascular arcade are the risk and prognostic factors for a poor outcome of visual acuity in BD patients.

  1. Uveitis and pseudouveitis presenting for the first time in Japanese elderly patients. Ikeda N, Hayasaka S, Hayasaka Y. Ophthalmologica. 2005 Sep-Oct;219(5):263-6.
    PURPOSE: To evaluate the clinical characteristics of uveitis or pseudouveitis presenting de novo in Japanese elderly patients. METHODS: We retrospectively examined 296 patients with uveitis or pseudouveitis who were treated at Toyama Medical and Pharmaceutical University Hospital. Patients were followed up for 1 week to 5 years (mean, 11 months). RESULTS: Of 296 patients, 82 (38 men and 44 women, 120 eyes) presented with their first episode of uveitis or pseudouveitis at the age of > or = 60 years. Forty patients had anterior uveitis, 2 had intermediate uveitis and the other 40 had posterior and/or pan-uveitis. Of 82 patients, 53 (64.6%) had idiopathic uveitis. The prevalence of Vogt-Koyanagi-Harada syndrome and Behcet disease in elderly patients was lower than that in younger patients. Complicated cataract was frequently seen in the eyes with uveitis. Most eyes with uveitis had poor visual acuity. CONCLUSIONS: The prevalence of Vogt-Koyanagi-Harada syndrome and Behcet disease in elderly patients was lower than that in younger patients.

  1. Clinical course and outcome of uveitis in children. Friling R, Kramer M, Snir M, Axer-Siegel R, Weinberger D, Mukamel M. J AAPOS. 2005 Aug;9(4):379-82.
    PURPOSE: We sought to evaluate the clinical course, complications, and visual outcome of pediatric uveitis. METHODS: Our sample included 38 consecutive children with uveitis treated between 1986 and 2002: 15 with idiopathic uveitis, 10 with Behcet disease, 9 with juvenile rheumatoid arthritis, 4 with other diagnoses. The last group was excluded from the analysis because of its small size. Appropriate ophthalmologic and laboratory examinations were obtained. Treatment consisted of topical, periocular, or systemic steroids with or without immunosuppression. RESULTS: Complications occurred in all 3 groups, although they tended to be more severe in the patients with juvenile rheumatoid arthritis. Visual acuity improved from presentation to final examination; the difference was statistically significant for the patients with idiopathic uveitis (P < 0.0005) and Behcet disease (P = 0.004). CONCLUSIONS: These findings support an intensive treatment approach to childhood uveitis. The visual prognosis is good even in patients with a severe clinical course.

  1. Clinical patterns and characteristics of uveitis in a tertiary center for uveitis in China. Yang P, Zhang Z, Zhou H, Li B, Huang X, Gao Y, Zhu L, Ren Y, Klooster J, Kijlstra A. Curr Eye Res. 2005 Nov;30(11):943-8.
    PURPOSE: To address the clinical pattern and characteristics of uveitis in a tertiary center for uveitis in China and compare the similarity and difference in the distribution of uveitis entities between China and other countries. METHODS: A retrospective study was performed on the patients with uveitis referred to the Zhongshan Ophthalmic Center from January 1996 to December 2003. The clinical data including category, etiology, gender, and the age of the patients at uveitis presentation were analyzed and compared with studies published previously from other countries. RESULTS: There were 902 male and 850 female patients in our series. The mean age of these patients at uveitis presentation was 33.8 +/- 16.5 years. Anterior uveitis (800, 45.6%) was the most common anatomical entity, followed by panuveitis (727, 41.5%), posterior uveitis (119, 6.8%), and intermediate uveitis (106, 6.1%). Further classification with the etiology criteria revealed 16 entities in anterior uveitis, with idiopathic anterior uveitis being the most common entity (473, 27.0%). Twelve entities were identified in panuveitis, of which Behcet disease (289, 16.5%) and Vogt-Koyanagi-Harada (VKH) syndrome (278, 15.9%) were the predominant ones. No specific entity was recognized in the intermediate uveitis group. Although a number of specific entities were identified in posterior uveitis, toxoplasmosis was noted in only two patients in this group. CONCLUSIONS: Idiopathic anterior uveitis, Behcet disease, and VKH syndrome are the most common entities of uveitis in China. Ocular toxoplasmosis, ocular histoplasmosis, and birdshot retinochoroidopathy are less common or absent in China.

  1. Visual aspects of Behcet's disease. Kansu T, Kadayifcilar S. Curr Neurol Neurosci Rep. 2005 Sep;5(5):382-8. (Review)
    Behcet's disease is a systemic vasculitis characterized by recurrent oral and genital ulcers and ocular inflammation. The disease may also involve the joints, skin, central nervous system, the lungs, and gastrointestinal tract. Ocular manifestations include anterior uveitis, posterior uveitis, macular edema, retinal vasculitis, conjunctival ulcers, optic neuropathy, papilledema, secondary glaucoma, cataract, and neovascularization of iris or retina. The cause of Behcet's disease is unknown but autoimmune and genetic factors are considered important in its pathogenesis. Promising new imaging techniques and treatments are available to provide better management for patients. This review focuses on recent data on visual aspects, pathogenesis, and the treatment of Behcet's disease.

  1. The prevalence of headache in Behcet's syndrome. Kidd D. Rheumatology (Oxford). 2006 May;45(5):621-3. Epub 2005 Dec 20.
    OBJECTIVES: Behcet's syndrome is an uncommon systemic disorder that involves the nervous system in 5% of cases. Headache may arise in conjunction with such complications but also appears to occur independently. We sought to define the prevalence of headache in an unselected group of patients with Behcet's syndrome, to characterize the headache syndromes and to identify what treatments are being used. METHODS: A questionnaire was sent to an unselected group of patients through their support group newsletter. RESULTS: The results showed a prevalence of recurrent headache in 82.5% of responders; the majority exhibited symptoms that fulfilled the International Headache Society criteria for migraine, with a higher than normal prevalence of visual or sensory aura of 52%. Using the Migraine Disability Assessment (MIDAS) score for disability in migraine, 62% of responders showed moderate or severe disability. Headache treatment was poor, the majority of sufferers resorting to over-the-counter remedies; preventative treatments had rarely been prescribed. CONCLUSIONS: Recurrent headache is very common in Behcet's syndrome, is poorly treated and is associated with disablement.

  1. Headache in Behcet's syndrome. Saip S, Siva A, Altintas A, Kiyat A, Seyahi E, Hamuryudan V, Yazici H. Headache. 2005 Jul-Aug;45(7):911-9.
    OBJECTIVE: To study the frequencies and characteristics of different headache types seen in patients with Behcet's syndrome (BS) in a large cohort of patients. BACKGROUND: Patients with BS may present with different neurological problems, related either directly or indirectly to the disease, with headache being the most common neurological symptom seen in this syndrome, as well as independent from neurologic involvement. METHOD: This study was carried out at the multidisciplinary Behcet outpatient clinic of the Behcet's Syndrome Research Center. Every fifth admitted patient who had fulfilled the International Study Group for Behcet's Disease classification criteria was recruited for this study. Each patient was interviewed by one of the examining neurologists through a semistructured questionnaire. In case of the presence of more than one headache type, the best defined one was diagnosed and evaluated. All patients received a complete neurological and physical examination. A chi(2) test was used to evaluate the differences between frequencies. One sample t-test was used to compare means. The prevalence rates of primary headaches were compared with results of the Turkish Headache Epidemiology study by using goodness-of-fit test. RESULTS: Two hundred and twenty-eight patients were studied. Headache was reported in 66.2% (151/228) of the study cohort. Primary headaches were seen in 38.6% of the patient population, which corresponds to 58% of BS patients with any type of headache. Tension-type headache (TTHA) and migraine were seen in 23.6% and 14.9% of the whole study cohort, respectively. In 5.2% of the study cohort, the headache was associated with neurological involvement and in 3.9% to uveal inflammation. A predominantly frontal, bilateral paroxysmal throbbing pain of moderate severity was reported in 18.4% of the study cohort who did not fulfill the criteria of the International Headache Society (IHS) for any of the primary headaches. It was commonly associated with exacerbations of the mucocutaneous symptoms of the syndrome. The neurological examination was normal in all of these patients. This type of headache was categorized as the nonstructural headache of Behcet. CONCLUSION: Headache is the most common neurological symptom seen in BS, both in patients with and without neurological involvement. The results of our study reveal that headache is seen in a majority of patients with neurological involvement due to BS and in a minority with uveal inflammation. The prevalence of migraine and TTHA are close to the population in general, but a nonstructural migrainous headache, which is commonly associated with exacerbations with some of the systemic symptoms of the syndrome, is noteworthy in patients with BS. This form of headache is not specific for this disorder, but may be explained by a vascular headache triggered by the immunomediated disease activity in susceptible individuals.

  1. CNS involvement occurs more frequently in patients with Behcet's disease under cyclosporin A (CSA) than under other medications--results of a retrospective analysis of 117 cases. Kotter I, Gunaydin I, Batra M, Vonthein R, Stubiger N, Fierlbeck G, Melms A. Clin Rheumatol. 2006 Jul;25(4):482-6. Epub 2005 Nov 1.
    The aim of this study was to evaluate the incidence of neurological manifestations of Behcet's disease (BD) in patients on cyclosporin A (CSA) compared with those on other medications. The records of 117 patients with BD who visited our hospital between 1990 and 2003 were reviewed with respect to symptoms and medication. All episodes of constant therapy prior to central nervous system (CNS) involvement were counted, and then the associations were analysed by the exact Fisher-Freeman-Halton test and adjusted for multiple tests by the Bonferroni-Holm method. We observed ten new cases of CNS manifestations in our patients with BD being regularly seen and treated in our tertiary care centre. The overall prevalence of neuro-BD in our patient group was 8.5%. In a retrospective analysis, the incidence of new-onset neurological disease (neuro-BD) in all patients with BD who regularly visited our hospital was significantly higher in patients on CSA than in those on other medications (6 of 21 vs 0 of 175 episodes, P<0.0001). This contrasts the obvious efficacy of CSA on extracerebral manifestations of BD, such as severe ocular disease, mucocutaneous lesions or arthritis. CSA exerts differential efficacy on various manifestations of BD. It is very effective for severe ocular and other moderate to severe manifestations of BD, but its efficacy for the prevention of neuro-BD seems to be inferior to that of other medications used in BD, such as azathioprine or interferon-alpha. The reasons for this are unclear, but the potential toxic effects of CSA on the CNS may be a predisposing factor for CNS vasculitis in BD.

  1. Diffusion-weighted imaging in chronic Behcet patients with and without neurological findings. Baysal T, Dogan M, Karlidag R, Ozisik HI, Baysal O, Bulut T, Sarac K. Neuroradiology. 2005 Jun;47(6):431-7. Epub 2005 May 26.
    Our aim was to investigate whether neurological impairment in chronic Behcet's disease (BD) patients with normal appearing brain can be assessed by means of diffusion-weighted imaging (DWI). The averaged apparent diffusion coefficient (ADC) values were calculated in 22 different radiologically normal appearing brain regions in 32 patients with and without neurological findings and 20 control subjects. The ADC values in bilateral frontal, temporal and occipital normal appearing white matter were significantly higher in the patient groups compared with the control subjects (p < 0.05). In these brain regions, DWI revealed differences in the ADC values between patients with neurological findings (including symptomatic and neuro-Behcet patients) and the asymptomatic patient group. The similarity of the ADC values of patients without symptoms to those of the control group allowed clear discrimination between patients with and without neurological findings. DWI may serve to assess subclinical neurological involvement in BD, even when structural changes are absent.

  1. Executive dysfunctions and depression in Behcet's disease without explicit neurological involvement. Erberk-Ozen N, Birol A, Boratav C, Kocak M. Psychiatry Clin Neurosci. 2006 Aug;60(4):465-72.
    This study aims to assess the executive functions and depression status in patients with Behcet's disease without explicit neurological involvement and to evaluate cognitive functions in this group of patients independent of accompanying depression. In the present study, 30 patients with Behcet's disease in the non-active phase of their illness and 30 healthy volunteers were included. In the evaluation of depression levels, Beck Depression Inventory was employed. The executive functions of the patients were evaluated by Wisconsin Card Sorting Test (WCST) and Stroop Test (ST). Beck Depression Inventory scores measured in the Behcet's disease group, in which no clinically serious depression was observed, were higher than the control group. Then, it was observed that the scores of neuropsychological tests of the Behcet's disease group were found to be significantly lower, especially in WCST, while significant relations were revealed between Beck Depression Inventory scores and WCST, ST scores. The authors' findings suggest that there may be factors other than depression causing executive impairment in patients with Behcet's disease without explicit neurological symptoms. Executive dysfunctions may occur in association with mild depressive states due to chronic disease stress or silent and/or future neurological involvement of Behcet's disease, especially in the frontal lobes.

  1. Changes in cerebral and ocular hemodynamics in Behcet's disease assessed by color-coded duplex sonography. Yilmaz S, Akarsu C. Eur J Radiol. 2006 Apr;58(1):102-9. Epub 2006 Feb 8.
    AIM: To quantify the cerebral and retrobulbar hemodynamics in Behcet's disease with and without ocular involvement and compared with that of healthy controls. MATERIALS AND METHODS: Of 51 people studied, 17 had Behcet's disease with ocular involvement, 17 had Behcet's disease without ocular involvement, and 17 were healthy controls. A single eye was examined in each patient. Peak systolic velocity (PSV), end-diastolic velocity (EDV), time-averaged maximum velocity (Tamax), and resistance index (RI) were evaluated in the ophthalmic (OA), posterior ciliary (PCA), central retinal (CRA) and middle cerebral artery (MCA). Additionally, the average blood flow velocities in the central retinal vein (CRV), and acceleration time (AT) and pulsatility index (PI) in the MCA were calculated. RESULTS: The mean EDV in the PCA was 25% lower and RI was higher in patients with ocular involvement of BD than in patients without involvement (p = 0.006 and p = 0.005, respectively) and in healthy controls (p = 0.003 and p = 0.004, respectively). Differences were smaller in comparisons of the CRA and absent on comparisons of the OA and MCA. The acceleration time of the MCA was significantly higher in patients with Behcet's disease than in healthy controls (p = 0.03). CONCLUSION: This study suggests that the flow hemodynamics in retrobulbar circulation has more altered Behcet's disease with ocular involvement than without ocular involvement and healthy control. Additionally, the cerebral hemodynamic might be affected in patients with Behcet's disease compared with healthy controls.

  1. Neurological symptoms of Adamantiades-Behcet's syndrome. Pearce JM. J Neurol Neurosurg Psychiatry. 2006 Aug;77(8):956-7. (Historical article)

  1. Neuro-Behcet's syndrome: differential diagnosis and management. Diri E, Espinoza LR. Curr Rheumatol Rep. 2006 Aug;8(4):317-22. (Review)
    Behcet's syndrome (BS) is a multisystem disease of unknown etiology, characterized by chronic relapsing cardinal symptoms of orogenital ulcers, uveitis, and different skin lesions. Its major pathologic feature is vasculitis. Neuro-Behcet's syndrome (NBS) is defined as a constellation of neurologic manifestations with characteristic neuropathologic findings, usually confirmed by ancillary investigations, in patients who meet the diagnostic criteria for BS. Neurologic manifestations of the syndrome are more common in male patients and have been reported to occur in anywhere from 5% to 50% of BS patients, depending on their geographical region. NBS primarily affects the central nervous system and includes parenchymal and nonparenchymal involvement. Peripheral neuropathy and myopathy are rare. Immunosuppression is widely used for treatment.

  1. Demyelination in rheumatic diseases. Theodoridou A, Settas L. J Neurol Neurosurg Psychiatry. 2006 Mar;77(3):290-5. (Review)
    Multiple sclerosis (MS) is a chronic inflammatory disease of the central nervous system (CNS) characterised by multifocal areas of demyelination in the white matter of the brain and spinal cord. Autoantibodies, for example antinuclear antibodies, can also be present. MS and other demyelinating processes, such as transverse myelitis and optic neuritis (which may be clinically isolated cases or be part of the clinical spectrum of MS), are sometimes difficult to differentiate from CNS involvement in systemic autoimmune diseases like systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS), Sjoegren's syndrome (SS), and Adamantiades-Behcet disease (BD). An acute isolated neurological syndrome presents the biggest diagnostic problem, since it is common in MS, but can also be the only feature or first manifestation in SLE, APS, SS, and BD. Indeed, the clinical presentation and lesions evidenced by magnetic resonance imaging may be similar.

  1. Difficulties in the differentiation of chronic inflammatory diseases of the central nervous system--value of cerebrospinal fluid analysis and immunological abnormalities in the diagnosis. Reske D, Petereit HF, Heiss WD. Acta Neurol Scand. 2005 Oct;112(4):207-13. (Review)
    OBJECTIVES: A number of neurological syndromes may be evoked by involvement of the nervous system due to systemic diseases such as lupus erythematodes, sarcoidosis, Behcet's disease and Sjogren's syndrome (SS) and may be confounded with another chronic inflammatory disease which is restricted to the central nervous system, e.g. multiple sclerosis (MS). Because of different treatment strategies, it is important to distinguish between these different autoimmune diseases. RESULTS: Neither clinical signs nor additional analyses such as serological findings or cerebrospinal fluid (CSF) analysis are able to differentiate between the diseases with certainty. Nevertheless, taking all findings together, diagnosis may be possible. CONCLUSION: Here we compare typical clinical and CSF findings in MS, neurosarcoidosis, neurolupus, neuro-Behcet and nervous system involving SS with special emphasis on those findings allowing differentiation of the respective diseases by reviewing the literature.

CARDIOVASCULAR DISEASE (including surgical treatment)
  1. Valvular surgery in Behcet's disease. Erentug V, Polat A, Bozbuga NU, Erdogan HB, Ozkaynak B, Akinci E, Yakut C. J Card Surg. 2006 May-Jun;21(3):289-91.
    BACKGROUND: Behcet's disease is a chronic inflammatory disease with a relapsing course. Behcet's disease affects many systems and causes hypercoagulability, and detection of an intracardiac mass in a Behcet patient should raise the question of an intracardiac thrombus. We analyzed our patients with Behcet's disease operated for valvular disease. METHODS: We operated three patients (one male and two females) who had been diagnosed as having Behcet's disease previously. Using mechanical bileaflet valves, aortic valve replacement in two and mitral valve replacement in the other patient were performed. Mechanical valve replacement was performed using pledgetted-interrupted sutures in the mitral procedure. Patients' steroid therapies were not interrupted and in the postoperative course, steroid was continued. No reoperations were needed. Anticoagulation with warfarin was instituted after the operation with the target of an international normalized ratio (INR) between 3 and 3.5. RESULTS: There was no mortality either early or late follow-up. Intraoperative and postoperative courses were uneventful. Two had ventricular arrhythmias. Total follow-up was 23.3 patient/years with a mean of 93.3 +/- 64.7 months. In the late follow-up, patient with the mechanical mitral valve experienced a cerebrovascular accident 40 months after the operation. Her echocardiographic examination gave a functional valve without any pathology. CONCLUSIONS: Surgeons should remember the hypercoagulable state in Behcet patients and strict anticoagulation protocols should be utilized. In the operations, bileaflet prostheses should be used.

  1. The surgical treatment of arterial aneurysms in Behcet disease: a report of 16 patients. Kalko Y, Basaran M, Aydin U, Kafa U, Basaranoglu G, Yasar T. J Vasc Surg. 2005 Oct;42(4):673-7.
    OBJECTIVE: The aim of this article is to report our experience in the surgical treatment of arterial aneurysms in patients with Behcet disease. METHODS: From October 2001 through May 2004, 18 arterial aneurysms were diagnosed in 16 Behcet patients. All patients were male. The patients ranged in age from 24 to 52 years (mean, 37.4 +/- 5.2 years). There were six abdominal aortic, three common femoral, two iliac, two popliteal, two superficial femoral, and two anterior tibial aneurysms and one subclavian artery aneurysm. All patients but four were in remission at the time of diagnosis. Those 4 patients received immunosuppressive therapy before the surgical intervention to induce remission. After hospital discharge, all patients were followed up regularly at 3-month intervals. The mean duration of follow-up was 17 +/- 4.2 months (range, 6-24 months). RESULTS: All patients underwent a successful surgical intervention. During the study period, we performed five aortic tube graft interpositions, two aortofemoral bypasses, one aortobifemoral bypass, three common femoral artery graft interpositions, and two femoropopliteal bypasses. The popliteal artery (n = 2), anterior tibial artery (n = 2), and subclavian artery (n = 1) aneurysms were repaired primarily. There was no in-hospital mortality. One patient with an abdominal aortic aneurysm had to undergo reoperation because of postoperative bleeding. The postoperative hospital stay was 8.5 +/- 4.3 days. Two patients were lost to follow-up. During the follow-up period, two false aneurysms of the common femoral artery were repaired with a graft interposition procedure. Another patient who had undergone an aortic tube graft interposition was readmitted 9 months later with an external iliac artery aneurysm. The external iliac artery was ligated through a retroperitoneal approach, and femorofemoral bypass was performed. In addition, one femoropopliteal interposition graft was occluded, without disabling ischemia. CONCLUSIONS: Although aneurysmal disease is rare in Behcet disease, it can complicate the clinical picture and cause life-threatening complications. We believe that the establishment of remission before the surgical intervention decreases the incidence of postoperative complications. Because recurrence at the site of anastomosis is possible, prolonged monitoring is required.

  1. Long-term outcome after surgical treatment of arterial lesions in Behcet disease. Hosaka A, Miyata T, Shigematsu H, Shigematsu K, Okamoto H, Ishii S, Miyahara T, Yamamoto K, Akagi D, Nagayoshi M, Nagawa H. J Vasc Surg. 2005 Jul;42(1):116-21.
    OBJECTIVE: Surgical treatment of arterial lesions associated with Behcet disease (BD) is often complicated by graft occlusion and recurrence of aneurysms. The purpose of this study was to clarify the long-term outcome of surgical intervention for arterial involvement in BD. METHODS: Ten patients with BD (9 men, 1 woman) who underwent surgical treatment for arterial aneurysms between 1980 and 2004 were included in the study. The age of patients at the first operation ranged from 36 to 69 years (mean, 50.4 +/- 9.0 years). The mean period between the onset of BD and that of arterial manifestations was 8.0 +/- 5.0 years. We retrospectively reviewed their postoperative courses, including survival, graft occlusion, formation of anastomotic false aneurysms, and the development of aneurysms at different sites. The Kaplan-Meier method was used to calculate the chronologic incidence of complications after surgery. RESULTS: The mean follow-up period was 133 +/- 92 months, ranging from 5 to 285 months. One patient died of rupture of a dissecting aortic aneurysm after undergoing several surgical interventions for multiple aneurysms. There were five graft occlusions among 21 grafts. The cumulative primary graft patency rate in the infrainguinal region was 83.9% at 3 years. Five anastomotic false aneurysms formed among 49 anastomoses between grafts and host arteries. The overall cumulative incidence of formation of anastomotic pseudoaneurysm was 12.9% at 5 and 10 years. All of them formed within 18 months after surgery. Development of new aneurysms in different arteries was observed in two patients. CONCLUSIONS: Early occurrence of anastomotic false aneurysm is characteristic of BD. Further investigation is necessary to establish effective postoperative treatment.

  1. Evaluation of myocardial perfusion in patients with Behcet's disease. Turkolmez S, Gokcora N, Alkan M, Gorer MA. Ann Nucl Med. 2005 May;19(3):201-6.
    AIM: To estimate the prevalence of silent myocardial ischemia (SMI) in patients with Behcet's disease (BD) and to identify a subgroup of patients at higher risk for the presence of SMI. MATERIALS AND METHODS: We evaluated 41 patients (mean age 42.8 +/- 12.3 years) with BD and 35 healthy control subjects. Treadmill exercise test and thallium-201 myocardial perfusion single photon emission computed tomography (SPECT) were performed in all subjects. Coronary angiography was performed in all patients with a diagnosis of SMI in Behcet's group. RESULTS: All subjects had normal resting electrocardiograms. Eight patients with BD (19.5%) had evidence of ischemia on exercise testing and myocardial perfusion SPECT. Only one SMI positivity (2.9%) was recorded in the control group. Significant coronary stenosis was not found with coronary angiography in the patients with a diagnosis of SMI in Becet's group. SMI positivity was recorded in 2 of 18 female patients (11%) and in 6 of 23 male patients (26.1%) with BD (p = 0.429). The mean duration of BD was 13.8 +/- 2.6 years in patients with SMI and 7 +/- 4.1 in patients without it (p < 0.001). Seven of the 8 patients (87.5%) with SMI had a duration of BD of greater than 10 years. CONCLUSIONS: The results of this study show that the prevalence of SMI is high in patients with BD. Based on our findings, screening with myocardial perfusion scintigraphy may be recommended for patients with duration of BD greater than 10 years.

  1. Morphologic evidence of subclinical atherosclerosis obtained by carotid ultrasonography in patients with Behcet's disease. Ozturk MA, Oktar SO, Unverdi S, Ureten K, Goker B, Haznedaroglu S, Sungur G, Reis KA, Onat AM. Rheumatol Int. 2006 Aug;26(10):867-72. Epub 2006 Jan 10.
    Subclinical atherosclerosis can be demonstrated by measuring the intima-media thickness (IMT) of the carotid arteries by high-resolution B-mode ultrasonography (US). Endothelial injury appears a key event in the atherogenesis. Endothelial cell activation and/or injury are the characteristic features of Behcet's disease (BD). In this study, we investigated morphologic evidence of subclinical atherosclerosis in the BD patients by using high-resolution B-mode US. Thirty-four patients with BD without arterial involvement (male/female 21/13; mean age 34.6+/-8.5 years) were individually matched to control subjects on the basis of age (within 2 years) and sex. Subjects with diabetes mellitus, hypertension, evidence of myocardial infarction or cerebrovascular disease, and patients on long-term steroids (i.e., >6 months) were excluded from the study. Mean IMT values of the right carotid arteries were 0.81+/-0.17 mm for patients with BD, and 0.54+/-0.13 mm for healthy controls (P<0.001). Mean IMT values of the left carotid arteries were 0.82+/-0.16 mm for patients with BD, and 0.55+/-0.12 mm for healthy controls (P<0.001). The overall prevalence of carotid atherosclerotic plaques was higher among the patients than the controls [prevalence of plaques were 17.6% (6/34) in BD patients and 0% in healthy controls, P<0.05]. In conclusion, our data indicate morphologic evidence of subclinical atherosclerosis in patients with BD.

  1. Increased thickness of the carotid artery intima-media assessed by ultrasonography in Behcet's disease. Keser G, Aksu K, Tamsel S, Ozmen M, Kitapcioglu G, Kabaroglu C, Killi R, Bayindir O, Doganavsargil E. Clin Exp Rheumatol. 2005 Jul-Aug;23(4 Suppl 38):S71-6.
    OBJECTIVE: Behcets disease (BD), is a unique systemic vasculitis, which affects almost all types and sizes of blood vessels. Carotid intima-media thickness (IMT) is an endothelial cell dysfunction (ECD) parameter which may also be associated with atherosclerosis. We aimed to search carotid IMT and plaque formation in BD, using high-resolution B-mode Doppler ultrasonography (USG). METHODS: We studied 114 BD patients (M/F: 68/46; mean age 38.15 +/- 9.44 years; disease duration 121 +/- 79 months), being followed up by Ege University Rheumatology Department. Age and sex-matched, 77 healthy controls, and as the disease control group 46 non-matched SLE patients were also included. Exclusion criteria for all the study participants were hypertension, hyperlipidemia, diabetes mellitus, obesity and history of cardiovascular or cerebrovascular disease. Comparison of the three groups were made by ANOVA and for post-hoc confirmation, Bonferoni test was used. RESULTS: The carotid IMT in BD (mean +/- SD, 0.55 +/- 0.14 mm) was significantly higher than in healthy controls (0.48 +/- 0.09 mm) (p = 0.004), but significantly lower than in SLE (0.66 +/- 0.24 mm) (p = 0.001). Likewise, plaque frequency in BD (5/114) was significantly higher than in healthy controls (0/77), but significantly lower than in SLE (8/46) (p < 0.001). CONCLUSION: Despite significantly higher carotid IMT and plaque frequency in BD compared with healthy controls, these parameters in BD were not as marked as in SLE. Less severe carotid artery abnormalities in BD, may partially explain why cardiovascular morbidity and mortality do not seem to be increased in BD, unlike in SLE.

  1. Assessment of aortic stiffness and ventricular diastolic functions in patients with Behcet's disease. Tunc SE, Dogan A, Gedikli O, Arslan C, Sahin M. Rheumatol Int. 2005 Aug;25(6):447-51. Epub 2005 Feb 23.
    BACKGROUND: Behcet's disease is a systemic vasculitis in which studies have given conflicting results about ventricular diastolic functions. However, tissue Doppler echocardiography has never been used in any of these studies. Aortic stiffness, a cardiovascular risk factor, may also precede ventricular dysfunction. OBJECTIVES: The aim of this study was to assess aortic stiffness and biventricular diastolic functions in patients with Behcet's disease. METHODS: A total of 26 patients with Behcet's disease (mean age; 33+/-10 years) and 20 age- and sex-matched controls (mean age; 33+/-7 years) were included. Aortic stiffness was evaluated by aortic strain and distensibility. Ventricular diastolic functions were evaluated with both conventional and tissue Doppler echocardiography. Mitral E and A wave, E/A ratio of E wave, deceleration time, and isovolumic relaxation time were calculated. RESULTS: There was no significant difference in diastolic Doppler parameters between patients and controls. Similarly, there was no significant difference in mitral annular E and A velocities between these two groups. Aortic strain in patients with Behcet's disease was found to be significantly less than in the controls (8.3+/-4.9% and 15.7+/-2.7% respectively, p<0.001). Aortic distensibility was also significantly low in patients with Behcet's disease when compared to controls (0.45+/-0.28 and 0.78+/-0.13 respectively, p<0.001). Beta index values were significantly high in Behcet's patients (7.23+/-5.93 and 2.69+/-0.55 respectively, p<0.001). CONCLUSION: No significant diastolic dysfunction was found in left and right ventricles in patients with Behcet's disease by using both conventional and tissue Doppler echocardiography. However, an increase in aortic stiffness was found, suggesting an inflammatory involvement of proximal aorta.

  1. Arterial distensibility as determined by carotid-femoral pulse wave velocity in patients with Behcet's disease. Kurum T, Yildiz M, Soy M, Ozbay G, Alimgil L, Tuzun B. Clin Rheumatol. 2005 Apr;24(2):134-8. Epub 2004 Sep 8.
    Behcet's disease (BD) is a chronic, multisystem disorder characterized by genital and oral aphthae, skin lesions, uveitis, and tendency to thrombosis. Pulse wave velocity (PWV) is an important factor in determining cardiovascular mortality and morbidity. It is an index of arterial wall stiffness and inversely related to the arterial distensibility. In this study we investigated the arterial distensibility in BD by PWV. We studied 14 patients with BD (18-44 years old, 10 men) and 28 healthy subjects (18-39 years old, 21 men) without known cardiovascular disease. Arterial distensibility was assessed by automatic carotid-femoral PWV measurement using the Complior Colson device. PWV is calculated from measurements of pulse transit time and the distance traveled by the pulse between two recording sites, according to the following formula: pulse wave velocity (m/s)=distance (m)/transit time(s). The mean ages, systolic blood pressure, diastolic blood pressure, pulse pressure, heart rate, and PWV of Behcet's disease and control subjects were 32.1+/-7.4 vs 27.9+/-6.1 years, 112.9+/-12.0 vs 108.7+/-10.0 mmHg, 72.1+/-10.7 vs 67.7+/-7.5 mmHg, 40.7+/-12.2 vs 41.0+/-10.7 mmHg, 74.1+/-10.2 vs 77.2+/-10.1 bpm, and 8.4+/-1.4 vs 8.5+/-1.1 m/s, respectively. Differences between all parameters studied were not found to be statistically significant (p>0.05). The carotid-femoral PWV, an index of arterial stiffness and a marker of atherosclerosis, is not increased in patients with BD compared with control subjects.

  1. Arterial wave reflections are associated with left ventricular diastolic dysfunction in Adamantiades-Behcet's disease. Ikonomidis I, Aznaouridis K, Protogerou A, Stamatelopoulos K, Markomihelakis N, Papamichael C, Kaklamanis P, Mavrikakis M, Lekakis J. J Card Fail. 2006 Aug;12(6):458-63.
    BACKGROUND: Adamantiades-Behcet's disease (ABD) is characterized by systemic vasculitis. We investigated whether aortic distensibility and arterial wave reflections are linked to left ventricular (LV) diastolic dysfunction in ABD. METHODS AND RESULTS: Eighty-two patients and 40 controls with similar atherosclerotic risk factors were examined by applanation tonometry of the radial artery (SphygmoCor) and echocardiography. Augmentation index (AI%) and arrival time (Deltat, ms) of reflected arterial waves as well as aortic distensibility (AoD) assessed by echocardiography were estimated. Doppler diastolic abnormalities were defined as proposed by the European Study Group on Diastolic Heart Failure by measurement of E/A ratio, isovolumic relaxation time, deceleration time, and flow propagation velocity. Patients had impaired central augmentation index (CAI), Deltat, and AoD compared with controls (P > .05). After adjusting for age, atherosclerotic risk factors, left ventricular mass, and medication the odds-ratio of AoD and CAI for left ventricular diastolic dysfunction was 0.664 (95%CI 0.449-0.982), P = .04, and 1.073 (95% CI 1.014-1.140), P = .001, respectively. The addition of CAI to the multivariable model including AoD significantly increased the power of the model for prediction of left ventricular diastolic dysfunction (-2 Log likelihood change = 18.8, P for change > .01). CONCLUSION: Augmentation index has a complementary value to aortic distensibility in the assessment of left ventricular diastolic dysfunction in ABD.

  1. Pressure wave reflections, central blood pressure, and aortic stiffness in patients with Adamantiades-Behcet's disease: a cross-sectional case-control study underlining the role of chronic corticosteroid treatment. Protogerou AD, Lekakis J, Ikonomidis I, Stamatelopoulos K, Aznaouridis K, Karatzis EN, Papamichael C, Markomihelakis N, Kaklamanis P, Mavrikakis M. Am J Hypertens. 2006 Jul;19(7):660-6; discussion 667-8.
    BACKGROUND: Adamantiades-Behcet's disease (ABD) is a multisystemic inflammatory/autoimmune disease involving both microcirculation and macrocirculation. Aortic stiffness index and aortic augmentation index (AI) are indices for the estimation of arterial stiffness and pressure wave reflections, respectively. The effect of anti-inflammatory and immunosuppressive drugs used in ABD on these indices is unknown. METHODS: In this cross-sectional study we examined 74 subjects with ABD (aged 40.1 +/- 12.5 years, 24 men) and 24 control subjects by using the noninvasive technique of radial artery applanation tonometry and pulse wave analysis for assessment of aortic AI by application of transfer functions. Echocardiography was used for assessment of aortic stiffness index. Classic cardiovascular (CV) risk factors, left ventricular and endothelial function of the brachial artery, as well as intima-media thickness of carotid artery, were also assessed. RESULTS: Corticosteroids were the only drug having a negative and independent effect on aortic AI, but not on aortic stiffness. Patients taking corticosteroids had lower aortic AI and central systolic blood pressure (BP), but not aortic stiffness and peripheral systolic BP, when compared to those without corticosteroids (21+/-14% v 12+/-14%, P < .050). Medication, traditional CV risk factors, and functional or structural CV parameters were all comparable among the two groups. The AI was similar between the control group and patients with ABD taking corticosteroids. CONCLUSIONS: The AI, but not aortic stiffness, is lower in patients with ABD taking corticosteroids compared to patients not taking corticosteroids and similar to the control group. These results imply a role of inflammation or immunomodulatory mechanisms in the regulation of pressure wave reflections.

  1. Echocardiographic findings in patients with Behcet's disease. Bozkurt A, Akpinar O, Uzun S, Akman A, Arslan D, Birand A. Am J Cardiol. 2006 Mar 1;97(5):710-5. Epub 2006 Jan 11.
    Case-controlled studies have shown that the prevalence of left ventricular (LV) diastolic dysfunction is higher in patients with Behcet's disease. However, there are no data evaluating the value of Doppler tissue imaging (DTI) in Behcet's disease. The aim of this study was to evaluate the cardiac involvement and the LV function at rest and at the end of isometric exercise by pulse-wave Doppler and DTI methods in patients with Behcet's disease. Fifty-four patients with Behcet's disease and 50 control subjects were studied. Dimensions of cardiac chambers, aortic root dimension, valvular abnormalities, and systolic function were similar in both groups. The mean E/A ratio was significantly lower in patients than controls (1.22 +/- .09 vs 1.36 +/- 0.30, p = 0.01). The E/A ratio was <1 in 12 patients (22%) and in 3 controls (6%) (p = 0.02). By DTI, no difference was found in the mean S-, e-, and a-wave velocities, and e/a ratio between the 2 groups. The e/a ratio was <1 in 13 patients (24.1%) and in 7 controls (14%) (p = 0.2) by DTI. There were no differences in the mitral pulse-wave Doppler and DTI parameters in patient and control groups at the end of exercise. The prevalence of cardiac pathology in Behcet's disease did not differ appreciably from the controls. In conclusion, the LV systolic and diastolic functions in the patients with Behcet's disease and controls were similar not only at rest but also at the end of exercise.

  1. Tissue Doppler velocities of the right and left ventricles and their association with C-reactive protein and homocysteine levels in Behcet's disease. Topal E, Ozdemir R, Aksoy Y, Acikgoz N, Ermis N, Sincer I, Akturk E, Ozcan B. Am J Cardiol. 2005 Dec 15;96(12):1739-42. Epub 2005 Nov 2.
    In the present study, we aimed to investigate whether tissue Doppler imaging could provide more information than standard echocardiography about left and right ventricular functions and their relations to homocysteine and C-reactive protein (CRP) levels in the course of Behcet's disease (BD). In the right ventricle, patients with BD had lower early diastolic tricuspid annular velocity and early diastolic/late diastolic tricuspid annular velocity ratios than the control group. Mitral and tricuspid annular systolic velocities did not significantly differ; however, diastolic velocities were correlated with duration of the disease. In addition, early/late diastolic mitral annular velocity ratio and early/late diastolic tricuspid annular velocity ratios showed an inverse relation with ocular involvement, serum homocysteine, and CRP levels in patients with BD.

  1. Characteristics of vascular involvement in Behcet's disease. Duzgun N, Ates A, Aydintug OT, Demir O, Olmez U. Scand J Rheumatol. 2006 Jan-Feb;35(1):65-8.
    OBJECTIVE: Behcet's disease (BD) is a multisystemic inflammatory disorder classified among the vasculitides, which can affect all types and sizes of blood vessels. Vascular involvement may be seen in 25-50% of BD patients. In this study, we examined the characteristics of vascular involvement in patients with BD. METHODS: One hundred and eighty patients with BD were included in the study. The diagnosis of vascular involvement was made on clinical signs, by Doppler ultrasonography and/or angiography using computed tomographic or magnetic resonance techniques where appropriate. Detailed clinical characteristics were recorded for each patient. RESULTS: Seventy-one patients (39.4%) had vascular involvement. In patients with vascular lesions, the frequency of male sex was significantly higher than in patients without vascular lesions (89.8% vs. 63.3%, respectively; p < 0.001). Of 71 BD patients with vascular involvement, 68 had venous lesions (95.8%). Three patients had arterial lesions without venous thrombosis. Eleven patients had arterial involvement with venous thrombosis. The most frequent type of vascular involvement was deep venous thrombosis in the lower extremities (n = 56, 78.9%). There was a significant association between deep venous thrombosis and superficial thrombophlebitis (r = 0.325, p < 0.01). Twenty-four patients (33.8%) had vena cava thrombosis and two had vena hepatica thrombosis. In patients with vascular involvement, the frequency of erythema nodosum was significantly higher (p = 0.001) and the frequency of ocular involvement was significantly lower (p < 0.05) than in patients without vascular involvement. CONCLUSION: Our study illustrates the frequency and significance of vascular involvement in BD.

  1. Pulmonary artery interventions: an overview. Pelage JP, El Hajjam M, Lagrange C, Chinet T, Vieillard-Baron A, Chagnon S, Lacombe P. Radiographics. 2005 Nov-Dec;25(6):1653-67. (Review)
    Interventional radiologists should be familiar with minimally invasive procedures used to treat various abnormalities of the pulmonary arteries. These well-established techniques, which obviate open surgery, are safe and effective when performed by an experienced interventionalist. Catheter-based thrombolysis with intrapulmonary arterial infusion of thrombolytic drugs, percutaneous thrombectomy, or embolus fragmentation can be performed in patients with life-threatening pulmonary embolism. Pulmonary artery stenoses, mainly encountered in patients with pulmonary vasculitis (as in Behcet disease or Takayasu arteritis), may be treated with balloon angioplasty and stent placement. Transcatheter embolization of pulmonary arteriovenous malformation is the standard treatment for hereditary hemorrhagic telangiectasia and is a very effective alternative to surgery to correct an aneurysm or pseudoaneurysm. In cases of hemoptysis that originates in the pulmonary artery, early diagnosis is mandatory for treatment with embolization. Percutaneous retrieval of foreign bodies from the heart or the pulmonary arteries and endovascular biopsy should also be part of the armamentarium of interventional radiologists.

  1. Topical granulocyte colony-stimulating factor for the treatment of oral and genital ulcers of patients with Behcet's disease. Bacanli A, Yerebakan Dicle O, Parmaksizoglu B, Yilmaz E, Alpsoy E. J Eur Acad Dermatol Venereol. 2006 Sep;20(8):931-5.
    BACKGROUND: Recurrent and painful ulcers of the oral mucosa and genital skin/mucosa are the most commonly observed manifestations in patients with Behcet's disease (BD). They affect patients' quality of life. Because of the effectiveness of granulocyte colony-stimulating factor (G-CSF) in wound healing, it may also be useful for the treatment of oral ulcers (OU) and genital ulcers (GU) of BD. OBJECTIVE: We aimed to determine the efficacy of topically applied G-CSF in the treatment of OU and GU of BD. METHODS: Seven patients with BD diagnosed according to the criteria of the International Study Group for Behcet's Disease were involved in the study. The patients were observed for 3 months before the study, and all occurrences were recorded during this period. Patients were given topical G-CSF for OU (4 x 120 microg/day, for 5 days) and/or GU (4 x 30 microg/day, for 5 days) and followed-up for 3 months after treatment. No concurrent disease-specific or immunosuppressive topical or systemic drugs were given during the study period. RESULTS: G-CSF treatment decreased the healing time and pain of OU and GU in six of seven patients compared with the pretreatment period. However, the effectiveness of the G-CSF treatment on OU and GU healing time and pain severity did not continue during the post-treatment period. CONCLUSIONS: G-CSF has beneficial effects on the healing duration and pain severity of OU and GU of patients with BD. However, given the high cost, impractical preparation and inability to cure the disease, G-CSF treatment should be chosen only in selected patients.

  1. The frequency of scarring after genital ulcers in Behcet's syndrome: a prospective study. Mat MC, Goksugur N, Engin B, Yurdakul S, Yazici H. Int J Dermatol. 2006 May;45(5):554-6.
    BACKGROUND: Although genital ulceration with or without scar formation is an important manifestation of Behcet's syndrome, formal data on the rate of genital scarring are lacking. OBJECTIVE: To study prospectively the frequency of genital scar formation after genital ulcers in patients with Behcet's syndrome. Methods One hundred and two patients (72 males and 30 females) with fresh genital ulcers presenting at a dedicated Behcet's syndrome outpatient clinic were followed for 6 weeks. The ulcer location, size, and frequency of scar formation were noted. All patients were treated with similar topical therapy. RESULTS: Two hundred and ten genital ulcers were followed in both sexes. The rate of scar formation was 66.2% (102/154) in male patients and 60.7% (34/56) in female patients. When genital ulcers were stratified as a function of size, large genital ulcers (> or = 1 cm in diameter) developed scars more frequently than did smaller ulcers (< 1 cm). The rate of scar formation in male patients with large genital ulcers was 89.4% (59/66) and with small ulcers was 48.9% (43/88) (P < 0.001). In females, all 19 large ulcers healed with scarring, and the same was true for 40.5% (15/37) of the small ulcers (chi(2) = 18.61, P < 0.001). CONCLUSION About two-thirds of genital ulcers in Behcet's syndrome leave scars. The rate of scar formation depends on the ulcer size.

  1. Can specific vessel-based papulopustular lesions of Behcet's disease be differentiated from nonspecific follicular-based lesions clinically? Boyvat A, Heper AO, Kocyigit P, Erekul S, Gurgey E. Int J Dermatol. 2006 Jul;45(7):814-8.
    BACKGROUND: Although papulopustular lesions are one of the diagnostic criteria for Behcet's disease, controversy exists as to the nature of these lesions. Specific vessel-based papulopustular lesions as well as nonspecific follicular lesions may be seen in patients with Behcet's disease. Some authors suggest that papulopustular lesions should be considered a positive criterion only if they exhibit a vessel-based neutrophilic reaction. OBEJECTIVE: To determine whether specific vessel-based papulopustular lesions can be differentiated clinically from nonspecific follicular lesions in patients with Behcet's disease. METHODS: Twenty-three papulopustular lesions in 20 patients with Behcet's disease were initially examined clinically by two dermatologists blind to each other's diagnosis. Biopsies taken from these lesions were examined by a pathologist unaware of the patient data. RESULTS: Leukocytoclastic vasculitis or perivascular infiltration was observed in 10 lesions, perifollicular and perivascular infiltration was noted in nine lesions, and perifollicular inflammation was seen in four biopsy specimens. Most of the lesions interpreted clinically as specific papulopustular lesions of Behcet's disease had predominantly perivascular infiltration or leukocytoclastic vasculitis; however, three papulopustular lesions evaluated by both observers as specific papulopustular lesions had only perifollicular inflammation, and one lesion diagnosed clinically as a nonspecific follicular eruption revealed perivascular neutrophilic reaction. Interobserver variance was noted in three papulopustular lesions. CONCLUSIONS: Clinical examination may not be sufficient to predict the dermatopathologic pattern in all lesions. Papulopustular lesions with no specific clinical and histopathologic features may create problems in the diagnosis of Behcet's disease.

  1. Histopathologic and direct immunofluorescence findings of the papulopustular lesions in Behcet's disease. Ilknur T, Pabuccuoglu U, Akin C, Lebe B, Gunes AT. Eur J Dermatol. 2006 Mar-Apr;16(2):146-50.
    Although papulopustular lesions are common in patients with Behcet's disease (BD), clinically they may not be differentiated from other diseases with papulopustular presentation such as acne vulgaris or folliculitis. Therefore, there is disagreement as to whether they should be used as a diagnostic criterion in BD. The aim of this study was to determine whether the histopathologic evaluation of the papulopustular lesions may assist in the diagnosis of BD. Eighteen patients with BD and 16 control patients consisting of eleven patients with bacterial folliculitis and five patients with acne vulgaris were included in the study. After the detailed histopathologic evaluation by two pathologists who were blinded to the clinical diagnoses, the histopathologic findings were classified into three patterns as follows; pattern I: vasculitis (lymphocytic or leucocytoclastic); pattern II: folliculitis and/or perifolliculitis; pattern III: superficial and/or deep perivascular, and/or interstitial dermatitis. In addition, direct immunofluorescence studies were performed in order to evaluate the deposition of IgM, IgG, IgA, C3, or fibrinogen in dermal blood vessels. 27.8% of the patients with BD (5 patients) revealed lymphocytic vasculitis, while none of the control group did; and the difference was found statistically significant (P=0.046). The rate of pattern II which included folliculitis and/or perifolliculitis was 50.0% in control patients and 16.7% in the patients with BD; and the difference was found statistically significant (P=0.038). No difference was found between the two groups with regard to pattern III or direct immunofluorescence findings (P>0.05). Our results indicate that only vasculitic changes can be useful when histopathological features of papulopustular lesions are to be employed as a diagnostic criterion in patients with suspected BD.

  1. Skin involvement and outcome measures in systemic autoimmune diseases. Albrecht J, Atzeni F, Baldini C, Bombardieri S, Dalakas MC, Demirkesen C, Yazici H, Mat C, Werth VP, Sarzi-Puttini P. Clin Exp Rheumatol. 2006 Jan-Feb;24(1 Suppl 40):S52-9. (Review)
    This paper focuses on skin manifestations that can be observed in autoimmune diseases such as rheumatoid arthritis (RA), Sjogren syndrome (SS), dermatomyositis (DM) and Behcet syndrome (BS). In RA the most widely recognized skin lesion is the rheumatoid nodule. Other cutaneous manifestations can be observed either non-specific or related to the disease itself and/or to the commonly used drugs. Cutaneous manifestations are considered one of the most typical extraglandular features of primary SS, generally they are distinguished in vasculitic and non vasculitic lesions. Among non-vasculitc lesions, skin dryness (xerosis) has been shown to be very common in pSS while vasculitis lesions include typically flat and palpable purpura and urticarial vasculits. In DM the skin manifestations are also frequent and include a heliotrope rash (blue-purple discoloration) on the upper eyelids with edema, a flat red rash on the face and upper trunk, and erythema of the knuckles with a raised violaceous scaly eruption (Gottron rash). The most frequent mucocutaneous finding in BS is aphthous stomatitis which can not usually be differentiated from idiopatic reccurrent aphthous stomatitis on clinical grounds. The most typical skin manifestations are nodular lesions, which are commonly seen in BS and may be due to panniculitis [erythema nodosum (EN)-like lesions] or superficial thrombophlebitis.

  1. Behcet's disease: treatment of mucocutaneous lesions. Alpsoy E. Clin Exp Rheumatol. 2005 Jul-Aug;23(4):532-9. (Review)
    Oral and genital ulcers, and cutaneous vasculitic lesions are considered hallmarks of the disease, and often precede other manifestations. Mucocutaneous lesions figure prominently in the presentation and diagnosis. Therefore, their recognition may permit earlier diagnosis and treatment, with beneficial results for prognosis. This review overviews the current state of knowledge regarding the therapeutic approaches including local and systemic agents for the treatment of mucocutaneous lesions of BD.

  1. Thalidomide: dermatological indications, mechanisms of action and side-effects. Wu JJ, Huang DB, Pang KR, Hsu S, Tyring SK. Br J Dermatol. 2005 Aug;153(2):254-73. (Review)
    Thalidomide was first introduced in the 1950s as a sedative but was quickly removed from the market after it was linked to cases of severe birth defects. However, it has since made a remarkable comeback for the U.S. Food and Drug Administration-approved use in the treatment of erythema nodosum leprosum. Further, it has shown its effectiveness in unresponsive dermatological conditions such as actinic prurigo, adult Langerhans cell histiocytosis, aphthous stomatitis, Behcet's syndrome, graft-versus-host disease, cutaneous sarcoidosis, erythema multiforme, Jessner-Kanof lymphocytic infiltration of the skin, Kaposi sarcoma, lichen planus, lupus erythematosus, melanoma, prurigo nodularis, pyoderma gangrenosum and uraemic pruritus. This article reviews the history, pharmacology, mechanism of action, clinical uses and adverse effects of thalidomide.

  1. How much helpful is the capsule endoscopy for the diagnosis of small bowel lesions? Ersoy O, Sivri B, Arslan S, Batman F, Bayraktar Y. World J Gastroenterol. 2006 Jun 28;12(24):3906-10.
    AIM: To assess the practically usefulness and diagnostic yield of this new method in a group of patients with suspected small bowel lesions. METHODS: Capsule endoscopic (CE) examination by using M2A capsule endoscope TM (Given Imaging, Yoqneam, Israel) was performed in thirty nine patients (26 males, 13 females) with suspected small intestinal lesions. The composing of the patients was as follows: obscure gastrointestinal bleeding in twenty three patients, known Crohn's disease in 6 patients, in whom CE was used to evaluate the severity and extension of the diseases, chronic diarrhea in 8 patients, abdominal pain in one patient and malignancy in one patient with unknown origin. RESULTS: In two patients CE failed. Different abnormalities were revealed in 26 patients overall. Detection rate of abnormalities was highest among patients with obscure gastrointestinal bleeding and the source of bleeding was demonstrated in 17 of 23 patients with obscure bleeding (73.9%). Entero-Behcet was diagnosed in two patients by CE as a source of obscure gastrointestinal bleeding. In 6 patients with known Crohn's disease, CE revealed better evaluation of the disease extension. In 3 of 8 (37.5%) patients with chronic diarrhea; CE revealed some mucosal abnormalities as the cause of chronic diarrhea. In a patient with unexplained abdominal pain and in a cancer patient with unknown origin, CE examination was normal. CONCLUSION: In our relatively small series, we found that capsule endoscopy is a useful diagnostic tool particularly in diagnosis of obscure gastrointestinal bleeding, chronic diarrhea and in estimating the extension of Crohn's disease.

  1. Indications for surgery in intestinal Behcet's disease. Kurata M, Nozue M, Seino K, Murata H, Sumita T, Katashi F. Hepatogastroenterology. 2006 Jan-Feb;53(67):60-3.
    BACKGROUND/AIMS: As advances in steroids and immunosuppressants made medical treatment first-line therapy for Behcet's disease, the criteria for elective surgery became unclear. The aim of this paper is to establish surgical criteria for Behcet's. METHODOLOGY: We reviewed the medical records of 8 consecutive Japanese patients who were diagnosed with intestinal Behcet's at Tsukuba University Hospital between 1976 and 2001. Records were examined for medical treatment including dose of steroids, and surgical treatment including length of resected intestine, incidence of recurrence, and the form and site of recurrence. RESULTS: Elective surgery was performed in cases where the preoperative steroid administration could not be reduced to under 40mg/day, once steroids exceeded 40mg/day to control symptoms and inflammation. Cases with emergency operations (perforated cases), the preoperative doses of steroids were not so high (0, 0, 10, 20mg/day). In all cases, the post-surgical steroid dose was reduced under 20mg/day. Three cases showed recurrence after surgery within 2 years. These cases also had an increase in steroid dosage at the time of recurrence, but have been controlled medically so far. CONCLUSIONS: Surgery should be strongly considered when the steroid dose exceeds 40mg/day.

  1. Comparison of Behcet's disease and recurrent aphthous ulcer according to characteristics of gastrointestinal symptoms. Rhee SH, Kim YB, Lee ES. J Korean Med Sci. 2005 Dec;20(6):971-6.
    Behcet's disease (BD) is a multisystemic chronic inflammatory disease. It is characterized by recurrent oral and genital ulcers, uveitis, skin lesions and other manifestations, including neurologic, vascular, joint, and gastrointestinal ulcers of variable severity. Recurrent aphthous ulcer (RAU) represents a very common, but poorly understood, mucosal disorder. If a patient of RAU without any other typical symptoms of BD has gastrointestinal symptoms, it is difficult to distinguish this RAU from true BD with gastrointestinal involvement. Because pathognomonic clinical features and tools are absent, the differential diagnosis of these two diseases relies on the characteristic clinical features and the judgement of an experienced physician. Sixty-five out of a total 960 RAU patients and forty-four of 556 BD patients with gastrointestinal symptoms between January 1996 and December 2003 participated in this study. All were evaluated with esophagogastroduodenoscopy and colonoscopy. Clinical, endoscopic and histopathologic findings were analyzed and ELISA tests were conducted to detect serum levels of ASCA and pANCA. No significant difference was found between the two groups. Differential diagnosis between RAU with gastrointestinal symptoms and BD with gastrointestinal involvement requires further prospective, large-scale study.

  1. High frequency of unusual gastric/duodenal ulcers in patients with Behcet's disease in Taiwan: a possible correlation of MHC molecules with the development of gastric/duodenal ulcers. Ning-Sheng L, Ruay-Sheng L, Kuo-Chih T. Clin Rheumatol. 2005 Sep;24(5):516-20. Epub 2005 Apr 23.
    The gastrointestinal (GI) involvement of Behcet's disease (BD) mainly affects the ileocecal region and colon. The gastroduodenal mucosa appears to be the least frequently involved segment of the gastrointestinal tract. The objective of this study was to assess the severity of gastric/duodenal involvement in BD patients in Taiwan. Behcet's disease was diagnosed according to the diagnostic criteria issued by the International Study Group for Behcet's Disease. We obtained and recorded clinical and laboratory data. A routine endoscopic examination with a urease test for Helicobacter pylori infection was arranged. Furthermore, HLA tissue typing was also performed by polymerase chain reaction with sequence-specific primers to evaluate the possible genetic loads associated with ulcer development. A total 28 BD patients, diagnosed at DaLin TzuChi hospital from 1999 to 2002, were enrolled in this study. The prevalence rate of gastric/duodenal ulceration was 43% (six patients had combined gastric and duodenal ulcers, three patients had simple gastric ulcers, and three patients had simple duodenal ulcers). No risk factors of nonsteroidal anti-inflammatory drug (NSAID) or H. pylori infection were found to be associated with gastrointestinal ulcers in our BD patients. All patients with peptic ulcers responded well to systemic steroids and immunosuppressant treatment in this preliminary observation. Furthermore, 7 of 12 gastric/duodenal ulcer patients (58%) carried an A2/B46/Cw1 or A11/B46/Cw1 genotype. Our data indicated that gastric/duodenal ulcers were a common manifestation in Chinese patients with BD in Taiwan in close association with the distinct genotypes of A2/B46/Cw1 or A11/B46/Cw1. A good response to systemic steroids, rather than conventional H2 blockers, might be due to downregulation of the vasculitis.

  1. Negative evaluation of a pathergy test in hepatitis B surface antigen carriers. Engin B, Guner OR. J Dermatol. 2006 Aug;33(8):547-9.
    The different cutaneous manifestations of hepatitis B virus infection have been described in different studies. We proposed to evaluate the frequency of skin pathergy positivity in hepatitis B surface antigen carriers. The study group consisted of 92 hepatitis B surface antigen carriers and 76 healthy individuals as a control group. The pathergy test was applied intradermally to both forearms of the patients and the control subjects. The results of the pathergy test were evaluated. Papule or pustule formation was noted as positive. Four and one of the 92 hepatitis patients and 76 control subjects, respectively, were positive for the pathergy reaction. Evidently, this pathergy test that shows non-specific skin hyper-reactivity cannot be used as a clinical marker to indicate hepatitis B surface antigen carriers. The test cannot be used to predict the cutaneous manifestations of hepatitis B infection either.

  1. Response to vaccination against hepatitis B in patients with Behcet's disease. Erkek E, Ayaslioglu E, Erkek AB, Kurtipek GS, Bagci Y. J Gastroenterol Hepatol. 2005 Oct;20(10):1508-11.
    BACKGROUND AND AIM: Hepatitis B virus infection is an important public health problem in Turkey. Although hepatitis B vaccination is regarded as safe and effective for the general population, recommendations for hepatitis B immunization in patients with Behcet's disease are not clear. The aim of the present study was to elucidate the response of patients with Behcet's disease to hepatitis B vaccination and to determine whether hepatitis B vaccination has any adverse effects on the course of the disease. METHODS: Thirteen patients with Behcet's disease and 15 healthy individuals were enrolled into a prospective study. All subjects received the 3-dose series of routine hepatitis B vaccine. Anti-hepatitis B surface response was evaluated 1-3 months after the third dose of vaccine. RESULTS: The responder rates for patient and control groups were 12/13 (92.8%) and 14/15 (93.8%), respectively. Statistical analysis showed no significant difference between the two groups in terms of both the responder rates and mean antibody titers. CONCLUSIONS: These preliminary findings might suggest that the majority of patients with Behcet's disease develop protective antibody response after hepatitis B vaccination and that the immune response against hepatitis B surface antigen is adequate, efficient and intact.

  1. Is there any association between hepatitis G virus (HGV), other hepatitis viruses (HBV, HCV) and Behcet's disease? Ozkan S, Toklu T, Ilknur T, Abacioglu H, Soyal MC, Gunes AT. J Dermatol. 2005 May;32(5):361-4.
    Infectious agents, especially viruses, have been implicated in the pathogenesis of Behcet's disease (BD). The aim of this study was to determine whether BD is associated with hepatitis viruses. In this study, the serological markers of hepatitis (HBsAg, anti-HBs, anti-HBc and anti-HCV) and viral nucleic acid (HGV-RNA) were studied in the sera of 35 patients, all of whom fulfilled the diagnostic criteria of the International Study Group for BD, and the results were compared with those of 36 healthy controls. The prevalences of HBsAg, anti-HBs, anti-HBc in BD patients were 2.9%, 45.7%, and 31.4%, respectively, which were not significantly different from those in healthy controls. None of the subjects in either group were found to be positive for anti-HCV. HGV-RNA was detected in two patients with BD and in none of the healthy controls. In conclusion, BD does not seem to be associated with hepatitis viral infections including hepatitis B, C, or G.

  1. Effect of Helicobacter pylori infection on deep vein thrombosis seen in patients with Behcet's disease. Senturk O, Ozgur O, Hulagu OS, Canturk NZ, Celebi A, Karakaya AT. East Afr Med J. 2006 Jan;83(1):49-51.
    OBJECTIVE: To investigate the role of homocysteine metabolism due to Helicobacter pylori infection on the development of deep vein thrombosis (DVT) in patients with Behcet's disease (BD). DESIGN: Prospective clinical study. SETTING: Teaching hospital. SUBJECT: Fifty-five patients with BD divided into groups, with DVT and without DVT, 19 healthy individuals and 18 patients with coronary artery disease (CAD) were enrolled into the study. INTERVENTIONS: Plasma homocysteine and Hp seropositivity were determined. RESULTS: There was significant Hp positivity in all groups (p > 0.05). Homocysteine levels were not significantly different for each group except patients with CAD (p > 0.05). CONCLUSION: There was no difference for frequency of Hp infection in all groups. We conclude that Hp does not influence DVT seen in BD via homocysteine metabolism, but the methinnin-loading test would be appropriate for enlighting patients whose fasting plasma homocysteine levels are found to be normal.

MORE PAPERS (including interventional trials) ...
  1. Oral zinc sulfate in the treatment of Behcet's disease: a double blind cross-over study. Sharquie KE, Najim RA, Al-Dori WS, Al-Hayani RK. J Dermatol. 2006 Aug;33(8):541-6.
    This was a randomized, controlled, double-blind trial of zinc sulfate in the treatment of Behcet's disease. Patients with Behcet's disease were recruited in this study between November 2001 and February 2003. A clinical manifestations index (CMI) was calculated for each patient. Serum zinc was estimated in all patients both at the beginning and monthly throughout the trial. Serum zinc levels were estimated from 30 healthy normal subjects matched for age and sex as a control group. Patients were randomly allocated to receive either 100 mg zinc sulfate or identical placebo tablet three times daily in a double-blind manner. After 3 months of starting treatment, patients were crossed over, that is, patients on placebo received zinc sulfate and vice versa. Mean serum zinc level in Behcet's disease patients was statistically significantly lower than mean serum zinc levels in healthy the control. In group A (started with zinc sulfate), the mean CMI started to decline directly after the first month of therapy with zinc sulfate to significantly lower levels. After shifting to placebo treatment in the fourth month, the mean of CMI started to rise again gradually but remained significantly lower than levels before therapy for the fourth and fifth months. In group B (started with placebo), the mean of CMI remained high for the first 3 months. After crossing over to zinc sulfate in the fourth month, the mean of CMI started to decrease after the fourth month. An inverse correlation between CMI and serum zinc level was found. No side-effects were seen in either group. In conclusion, zinc sulfate was found to be a good option in the treatment of Behcet's disease.

  1. A double-blind trial of depot corticosteroids in Behcet's syndrome. Mat C, Yurdakul S, Uysal S, Gogus F, Ozyazgan Y, Uysal O, Fresko I, Yazici H. Rheumatology (Oxford). 2006 Mar;45(3):348-52. Epub 2005 Nov 1.
    OBJECTIVES: Corticosteroids are widely used in Behcet's syndrome despite the absence of controlled studies. We assessed the effect of depot corticosteroids primarily for genital ulcers and secondarily for the other mucocutaneous manifestations of Behcet's syndrome. METHODS: We randomized 86 patients who had active disease with genital ulcers to receive either intramuscular corticosteroid injections (40 mg methylprednisolone acetate) or placebo every 3 weeks for 27 weeks. RESULTS: Seventy-six patients (88%) completed the treatment. There were no significant differences in the mean number of genital and oral ulcers, or folliculitis between groups. The mean number of erythema nodosum lesions was less in the corticosteroid group as a whole (P = 0.0046); subgroup analyses revealed that this was significant for females (P = 0.0148) but not for males (P = 0.1). CONCLUSION: Low-dose depot corticosteroids did not have any beneficial effect on genital ulcers. However, it was useful in controlling erythema nodosum lesions, especially among the females.

  1. Colchicine and benzathine penicillin in the treatment of Behcet disease: a case comparative study. Al-Waiz MM, Sharquie KE, A-Qaissi MH, Hayani RK. Dermatol Online J. 2005 Dec 1;11(3):3.
    Behcet disease is a chronic relapsing disease characterized by multiple signs and symptoms such as recurrent orogenital ulceration, eye involvement, skin manifestations, and other systemic involvement. Multiple therapeutic modalities have been used to treat Behcet disease. These agents act through different mechanisms and are associated with a variety of side effects. We performed a case-comparative study to evaluate efficacy of combined colchicine and benzathine penicillin in the treatment and prophylaxis of Behcet disease. Sixty-six patients who fulfilled the international study group criteria for diagnosis of Behcet disease were included. The patients were divided into three groups: group 1 (20 patients) received 1.2 Mu benzathine penicillin injection monthly; group 2 (21 patients) received two tablets of colchicine daily (each tablet contained 0.5 mg); and group 3 (25 patients) received both 1.2 Mu benzathine penicillin injection monthly and two tablets of colchicine daily. Each patient was followed up monthly for 5 months, 4 months on treatment and 1 additional month followup. The clinical manifestation index (CMI), the numerical sum of the clinical features, was calculated for each patient initially and then monthly. Pathergy test was performed for each patient monthly. The CMI was reduced by colchicine and benzathine penicillin treatment, and the reduction was highly significant. The reduction in the CMI remains satisfactory and good for 1 month after stopping the treatment. When each colchicine and benzathine Penicillin are used alone the index is also reduced significantly, but this reduction is much less than when both drugs are used together and there is also rapid and earlier relapse. Based on our findings, the combination of colchicine and benzathine penicillin appears to be of greater efficacy in the treatment of Behcet disease than the use of either drug alone.

  1. Prophylactic calcium and vitamin D treatments in steroid-treated children with nephrotic syndrome. Bak M, Serdaroglu E, Guclu R. Pediatr Nephrol. 2006 Mar;21(3):350-4. Epub 2005 Dec 29.
    Steroid treatment has several side effects, including the deterioration of the bone and mineral metabolism in children with nephrotic syndrome. This randomized prospective study was conducted to determine the effects and prophylactic role of calcium plus vitamin D treatment on bone and mineral metabolism in children receiving prednisolone treatment. 40 children (27 boys and 13 girls) with NS (18 new onset and 22 relapsing) were included in the study. Their mean age was 4.6+/-1.8 years. All patients received prednisolone treatment (2 mg/kg/day for 4 weeks followed by alternate days at the same dose for 4 weeks). The patients were randomized into treatment (vitamin D 400 IU plus calcium 1 g daily) and non-treatment groups. Bone mineral density, serum Ca, P, alkaline phosphatase and urinary Ca and P excretions were analyzed at the beginning and 2 months after the treatment. The XR36 Norland device was used for bone mineral density analysis. Bone mineral density was significantly decreased in both the treatment (0.54+/-0.15 to 0.51+/-0.1 g/cm(2), P =0.001) and non-treatment (0.52+/-0.18 to 0.45+/-0.16 g/cm(2), P <0.001) group. But the percentage of bone mineral density decrease was found to be significantly lower in the treatment group than in the non-treatment group (4.6+/-2.1% vs. 13.0+/-4.0%, respectively; P <0.001). Serum calcium and urinary calcium excretion increased in the treatment group (8.0+/-1.0 to 10.0+/-0.5 mg/dl and 1.1+/-0.5 to 3.2+/-1.0 mg/kg/day) and non-treatment group (8.1+/-0.8 to 10.0+/-0.6 mg/dl and 1.4+/-0.9 to 3.8+/-3.3 mg/kg/day) after prednisolone treatment (P <0.001). Steroid treatment decreases bone mineral density in children with nephrotic syndrome. Vitamin D plus calcium therapy at the current doses reduces but does not completely prevent bone loss, with no additional adverse effects.

  1. Sonographic evaluation of the tendons in familial Mediterranean fever and Behcet's disease. Ozcakar L, Onat AM, Ureten K, Cetin A, Kiraz S, Ertenli I, Calguneri M, Akinci A. Joint Bone Spine. 2006 Oct;73(5):514-7. Epub 2006 Mar 31.
    OBJECTIVES: There have been some studies on the burden of certain rheumatological disorders on the tendons; however, patients with familial Mediterranean fever (FMF) and Behcet's disease (BD) have not, up to now, been studied in this regard in the literature. Thus, this current study centers on ensuing changes in the tendons of these patients. METHODS: The study comprised 32 patients with FMF (13 male, 19 female), 31 with BD (18 male, 13 female) and 35 control subjects (17 male, 18 female). Sonographical evaluations were performed from the triceps, quadriceps and Achilles tendons on the non-dominant extremities of the individuals using a linear array probe of 8-16 MHz. RESULTS: The mean triceps tendon (TT) thickness value of FMF patients was greater than that of BD patients (P=0.03) or the controls' (P=0.02). The mean quadriceps tendon (QT) thickness value of BD patients was greater than that of FMF patients (P=0.00) or the controls' (P=0.01). The mean Achilles tendon (AT) thickness value of BD patients was greater than that of controls' (P=0.05) only. There was not any difference between the tendon thickness measurements of either group of patients with and without arthritic involvement. CONCLUSION: Our first and preliminary findings pertaining to increased tendon thicknesses in FMF (TT) and BD (QT and AT) patients should be complemented with future histological and clinical studies. The functional relevance of tendon thickening, its probable reflection on the rupture risk and the role of each disease related contributing factor remain to be uncovered.

  1. Arthropathy, quality of life, depression, and anxiety in Behcet's disease: relationship between arthritis and these factors. Gur A, Sarac AJ, Burkan YK, Nas K, Cevik R. Clin Rheumatol. 2006 Jul;25(4):524-31. Epub 2006 Apr 26.
    We aimed to determine arthritis frequency, quality of life (QoL), anxiety, and depression levels in Behcet's disease (BD) and thereby the effect of joint involvement on QoL, anxiety, and depression. Sixty-three patients diagnosed with BD according to the International Study Group Diagnostic Criteria and 45 healthy subjects as control were included in the study. All patients were evaluated in terms of clinical findings, the number and site of the joints involved, sacroiliac joint involvement, pain intensity, QoL, anxiety, and depression scale. The instruments used were Beck Depression Inventory (BDI) for depression, State-Trait Anxiety Inventory (STAI-T) for anxiety, and Health Assessment Questionnaire (HAQ) and Nottingham Health Profile (NHP) for health status and QoL. Joint involvement was seen in 41.3% of the patients. The most characteristic form was asymmetric oligoarthritis, which was seen in 65.3% of the patients. The joints most commonly involved were found to be in the wrist (53.8%) and knee (50%). Sacroiliitis was found at a rate of 38.1%. In BD patients without arthritis, rates of men (p=0.004) and genital ulcer (p=0.001) were higher, and rates of erythema nodosum (p=0.001) and human leukocyte antigen B5 positivity (p=0.023) were less than those of BD patients with arthritis. Pain intensity (p=0.000), NHP (p=0.004), and HAQ (p=0.003) scores were significantly higher in BD patients with arthritis than those without arthritis. Pain intensity, NHP, BDI, and STAI-T scores were significantly higher in BD patients without arthritis than in healthy controls, while pain intensity, NHP, HAQ, BDI, and STAI-T scores were significantly higher in BD patients with arthritis than in healthy controls (p=0.000). In conclusion, our study establishes that arthropathy is one of the common manifestations of BD. Arthritis in BD affects considerably patients' pain levels and QoL. A multistep approach is required for these patients. We are of the opinion that this approach may also improve the QoL of these patients and prevent the activation of the disease.

  1. Peripheral insulin resistance in patients with Behcet's disease. Erdem H, Dinc A, Pay S, Simsek I, Turan M. J Eur Acad Dermatol Venereol. 2006 Apr;20(4):391-5.
    In this study, we examined peripheral insulin resistance in patients with Behcet's disease (BD) characterized by chronic inflammation and endothelial dysfunction. Fourteen patients with BD and 15 healthy controls were recruited to the study. Insulin resistance was investigated by the hyperinsulinaemic-euglycaemic glucose clamp technique. BD patients displayed an enhanced rate of insulin resistance compared to healthy controls (P = 0.014). The insulin sensitivity (M), measured as the glucose utilization rate under steady-state conditions of euglycaemia, was significantly decreased (P = 0.001) in BD patients compared to the controls (4.09 +/- 0.16 vs. 5.60 +/- 0.27 mg/kg/min). The C-reactive protein (CRP) level, but not the erythrocyte sedimentation rate (ESR), was significantly related to the presence of insulin resistance (CRP: r(s) = 0.589, P = 0.27; ESR: r(s) = 0444, P = 0112), whereas no relationship was found between the M-value and ESR or CRP. We conclude that patients with BD exhibit peripheral insulin resistance; this could be explained as the diverse consequences of inflammation and endothelial dysfunction in BD.

  1. Fibrinolytic activity and d-dimer levels in Behcet's syndrome. Yurdakul S, Hekim N, Soysal T, Fresko I, Bavunoglu I, Ozbakir F, Tabak F, Melikoglu M, Hamuryudan V, Yazici H. Clin Exp Rheumatol. 2005 Jul-Aug;23(4 Suppl 38):S53-8.
    OBJECTIVE: Thrombophlebitis occurs in a third of patients with Behcet's syndrome (BS). The thrombotic tendency in BS has been studied with inconclusive results perhaps due to the inadequate numbers of patients studied during the acute phase of the thrombosis as well as the lack of appropriate diseased controls. We have studied tissue-type plasminogen activator (t-PA) and its inhibitor (PAI-1), and d-dimer levels in BS patients with and without thrombosis both in the acute and chronic phases along with suitable diseased and healthy controls. METHODS: t-PA and PAI-1 were studied by ELISA and d-dimer by semiquantitative latex agglutination slide test in 30 BS patients without deep vein thrombosis (DVT), 10 BS with acute DVT (ADVT), 25 BS with chronic DVT, 27 with ankylosing spondylitis, 26 diffuse systemic sclerosis, 15 patients with ADVT due to other causes, 10 patients with sepsis, and 23 healthy controls. RESULTS: The t-PA levels in BS with ADVT were significantly lower than those in patients with ADVT due to other causes (7.4 +/- 6.2 vs. 13.4 +/- 6.3, P = 0.027) while PAI-1 levels did not show significant differences between the groups (P = 0.60). The numbers of patients with d-dimer levels of > or = 0.5 microg/ml in BS with ADVT were similar to those found in patients with ADVT due to other causes (9/10 vs. 14/14). CONCLUSION: The relatively low t-PA levels point to a defect in fibrinolysis in BS. d-dimer levels are increased in the acute phase of thrombosis in BS.

  1. Thrombophilic risk factors and homocysteine levels in Behcet's disease in eastern Spain and their association with thrombotic events. Ricart JM, Vaya A, Todoli J, Calvo J, Villa P, Estelles A, Espana F, Santaolaria M, Corella D, Aznar J. Thromb Haemost. 2006 Apr;95(4):618-24.
    Behcet's disease (BD) is a chronic inflammatory disorder in which thrombosis occurs in about 30% of patients. The prothrombotic mechanisms are unknown. Thrombophilic defects and hyperhomocysteinaemia may be involved in the pathogenesis of thrombotic events, although results have been controversial. Moreover, no information is available on this issue for eastern Spain. We studied the prevalence of inherited and acquired thrombophilic risk factors in 79 patients with BD (43 men, 36 women) who had (n = 23) or did not have (n = 56) thrombosis, and in 84 healthy control subjects (42 men, 42 women). Risk factors examined were antithrombin, protein C and protein S levels, factor V Leiden, the prothrombin G20210A mutation, the methylenetetrahydrofolate reductase C677T polymorphism, and acquired thrombophilic risk factors, including anticardiolipin antibodies, lupus anticoagulant, and serum homocysteine levels. There were no differences between patients and controls in any of the parameters studied. When we studied BD patients with and without thrombotic events, the only thrombophilic defect that differed was the prothrombin G20210A mutation: Three out of 23 patients with thrombosis were carriers, compared with none of 56 patients without thrombosis (p = 0.022). Two of the three carriers developed catastrophic or recurrent thrombotic episodes; one was a homozygous carrier of the G20210A prothrombin mutation and the other was doubly heterozygous for the G20210A prothrombin mutation and factor V Leiden. A meta-analysis demonstrated an association of factor V Leiden and prothrombin mutation with thrombosis in BD. When studies from Turkey were excluded from the meta-analysis, only the prothrombin G20210A mutation was associated with thrombosis.

  1. Imaging infection with 18F-FDG-labeled leukocyte PET/CT: initial experience in 21 patients. Dumarey N, Egrise D, Blocklet D, Stallenberg B, Remmelink M, del Marmol V, Van Simaeys G, Jacobs F, Goldman S. J Nucl Med. 2006 Apr;47(4):625-32.
    The aim of this study was to assess the feasibility and the potential role of PET/CT with (18)F-FDG-labeled autologous leukocytes in the diagnosis and localization of infectious lesions. METHODS: Twenty-one consecutive patients with suspected or documented infection were prospectively evaluated with whole-body PET/CT 3 h after injection of autologous (18)F-FDG-labeled leukocytes. Two experienced nuclear medicine physicians who were unaware of the clinical end-diagnosis reviewed all PET/CT studies. A visual score (0-3)-according to uptake intensity-was used to assess studies. The results of PET/CT with (18)F-FDG-labeled white blood cell ((18)F-FDG-WBC) assessment were compared with histologic or biologic diagnosis in 15 patients and with clinical end-diagnosis after complete clinical work-up in 6 patients. RESULTS: Nine patients had fever of unknown etiology, 6 patients had documented infection but with unknown extension of the infectious disease, 4 patients had a documented infection with unfavorable evolution, and 2 patients had a documented infection with known extension. The best trade-off between sensitivity and specificity was obtained when a visual score of >or=2 was chosen to identify increased tracer uptake as infection. With this threshold, sensitivity, specificity, and accuracy were each 86% on a patient-per-patient basis and 91%, 85%, and 90% on a lesion-per-lesion basis. In this small group of patients, the absence of areas with increased WBC uptake on WBC PET/CT had a 100% negative predictive value. CONCLUSION: Hybrid (18)F-FDG-WBC PET/CT was found to have a high sensitivity and specificity for the diagnosis of infection. It located infectious lesions with a high precision. In this small series, absence of areas with increased uptake virtually ruled out the presence of infection. (18)F-FDG-WBC PET/CT for infection detection deserves further investigation in a larger prospective series.

  1. Oral health related quality of life is affected by disease activity in Behcet's disease. Mumcu G, Inanc N, Ergun T, Ikiz K, Gunes M, Islek U, Yavuz S, Sur H, Atalay T, Direskeneli H. Oral Dis. 2006 Mar;12(2):145-51.
    OBJECTIVES: This study aimed to investigate oral and general health related quality of life (QoL) in patients with Behcet's Disease (BD) and to assess the performance of Turkish versions of oral health related quality questionnaires. SUBJECTS AND METHODS: Ninety-four BD patients, 24 patients with recurrent aphthous stomatitis (RAS), 113 healthy controls (HC) and 44 dental patients were investigated. QoL was assessed by oral health impact profile-14 (OHIP-14), oral health related quality of life (OHQoL) and short form-36 (SF-36) questionnaires. RESULTS: OHQoL, OHIP-14 and SF-36 subscale scores were significantly worse in patients with BD compared with those in HC (P < 0.05). Both OHIP-14 and OHQoL scores were significantly worse in active patients compared with inactives in BD and RAS (P < 0.05). Scores of SF-36 Role physical, Role emotional and Vitality were also lower in active patients than in inactives in BD (P < 0.05). Scores of OHIP-14 and OHQoL were significantly worse in patients treated with colchicine compared with those treated with immunosuppressives (P < 0.05). CONCLUSIONS: Both oral and general QoL was impaired in BD and associated with disease activity and treatment modalities. Translated Turkish versions of OHIP-14 and OHQoL were also observed to be valid and reliable questionnaires for further studies.

  1. Behcet's disease and hereditary periodic fever syndromes: casual association or causal relationship? Espinosa G, Arostegui JI, Plaza S, Rius J, Cervera R, Yague J, Font J. Clin Exp Rheumatol. 2005 Jul-Aug;23(4 Suppl 38):S64-6.
    OBJECTIVE: Mutations in the MEFV and the type 1 TNF receptor (TNFRSF 1A) genes have recently been linked to familial Mediterranean fever (FMF) and TNF receptor-associated periodic syndrome (TRAPS), respectively. A higher prevalence of Behcet's disease (BD) among FMF patients has been described compared to the general population. The aim of this study was to evaluate whether FMF TRAPS and BD could be genetically related. METHODS: We screened a cohort of 50 BD patients and 100 healthy subjects for the common MEFV and TNFRSF 1A mutations. An initial screening of exons 10 and 2 of the MEFV gene and exon 4 of the TNFRSF 1A was performed in all chromosomes. RESULTS: The heterozygous MEFV mutation (K695R) was found in one (2%) BD patient. Analysis for FMF mutations in the control group revealed that 5 (5%) individuals bore MEFV gene mutations (3 were heterozygous for the E148Q and 2 were heterozygous for the A744S). At codon 202, there were no differences in allele frequencies between BD and control population: 73%R 27%Q in the BD patients vs 75%R 25%Q in controls. Concerning mutations in the TNFRSF 1A gene, the R92Q mutation was present in heterozygous state in one (2%) BD patient and in 4 (4%) controls without differences between allele frequencies: 99%R 1%Q in BD patients vs 98%R 2%Q in controls, respectively. There was no association between the clinical manifestations of BD patients and the presence of a particular polymorphism or a mutation. CONCLUSIONS: Neither FMF nor TRAPS are genetically associated with BD in our cohort of Spanish patients.

  1. Behcet's disease associated with malignancies. Report of two cases and review of the literature. Kaklamani VG, Tzonou A, Kaklamanis PG. Clin Exp Rheumatol. 2005 Jul-Aug;23(4 Suppl 38):S35-41.
    OBJECTIVE: To investigate the incidence of malignancies in a cohort of Behcet's disease patients and review the world literature. METHODS: Our database of 128 patients was searched and the age standardized rate (ASR) for cancer was calculated. Furthermore, we performed a MEDLINE search from 1970 through 2003, as well as, a search in the proceedings of international conferences for cases of malignancies associated with Behcet's disease. RESULTS: Two of our 128 patients with Behcet's disease were found to have solid tumors. One male had lung cancer and the other female had kidney cancer. The ASR for cancer cases in our population was investigated and it was found to be 1,600 per 100,000 in 10 years. The ASR for cancer cases in Greece according to WHO is 272.51 per 100,000 per year and therefore 2,725 per 100,000 in 10 years. In the world literature 112 cases of malignancies associated with Behcet's disease were found: Sixty five cases were of male patients and 46 of female with 1 case of unknown gender. The solid malignancies associated with Behcet's disease included cases of bladder, breast, uterus, thyroid and stomach cancer, whereas haematological malignancies included leukemia, myelodysplastic syndrome, lymphoma, multiple myeloma, Hodgkin's disease and lymphosarcoma. The treatment administered in these patients with their disease is also reported. CONCLUSION: The age standardized rate of cancer in our population was lower than that of the general population in Greece, although the difference was not statistically significant. However, there is discrepancy in the world literature and the possibility of development of malignancies in Behcet's disease patients should not be ignored.

  1. Quality of life and life satisfaction in patients with Behcet's disease: relationship with disease activity. Bodur H, Borman P, Ozdemir Y, Atan C, Kural G. Clin Rheumatol. 2006 May;25(3):329-33. Epub 2005 Nov 3.
    Quality of life (QoL) and life satisfaction (LS) are important outcome factors in chronic inflammatory conditions such as Behcet's disease (BD). The aim of this study was to investigate QoL and LS in patients with BD and determine the relationship with disease activity. Forty-one patients with BD and 40 control subjects were involved in the study. Demographic properties were obtained. Disease activity was assessed by Turkish version of BD Current Activity Form (BDCAF) in BD patients. QoL and psychological well-being were assessed by Nottingham Health Profile (NHP) and Life Satisfaction Index (LSI), respectively, in both patients and control groups. The related disease activity measures of QoL and LS were determined. Twenty-two male and 19 female BD patients with a mean age of 33.3+/-9.3 years and 20 male and 20 female control subjects with a mean age of 33.3+/-4.1 years were involved. According to BDCAF, no patient had central nervous system involvement. Thirty-four patients had headache, 33 patients had fatigue, 30 patients had articular involvement, 29 had mucocutaneous lesions, 27 had gastrointestinal involvement, 21 patients had ocular involvement, and 7 patients had vascular involvement. The scores of all dimensions of NHP were significantly higher and the mean score of LSI was significantly lower in BD patients than in control subjects (p<0.001). Correlation analysis indicated that the scores of fatigue, joint involvement, and oral ulcers were the most related factors for physical domains of NHP, whereas joint involvement and genital ulcers were the most related activity measures for psychosocial subscales of NHP. LS was found to be most related with the scores of patient's and physician's impression of disease activity and joint involvement. In conclusion, patients with BD have impaired QoL and disturbed psychological well-being. Current management strategies focusing on fatigue, arthralgia, mucocutaneous lesions, and efforts to measure psychosocial aspects and symptoms of the patients by their point of view will help to improve QoL and raise the LS in patients suffering from BD.

  1. Behcet's disease combined with various types of fistula. Chung HJ, Goo BC, Lee JH, Bang D, Lee KH, Lee ES, Lee S. Yonsei Med J. 2005 Oct 31;46(5):625-8.
    Behcet's disease (BD) is a chronic, relapsing, multisystem disorder, characterized by recurrent oral ulcer, genital ulcers, eye lesion, and skin lesion. The underlying pathology is nonspecific vasculitis of all vessel sizes, and severe vasculitis can result in fistula formation of neighboring tissues due to a necrotic process. Herein, eleven cases of BD combined with fistula are presented. In the present study, various types of fistula were associated; enterocutaneous fistula in six patients, and rectovaginal fistula in two. The other three patients showed aortoduodenal fistula, urethrovaginal fistula and urethrocutaneous fistula. They were treated with a corrective operation, but the prognoses were poor due to frequent relapses.

  1. Pulmonary vasculitis in behcet disease: a cumulative analysis. Uzun O, Akpolat T, Erkan L. Chest. 2005 Jun;127(6):2243-53.
    STUDY OBJECTIVES: The aims of this study were to investigate the frequency of pulmonary problems in Behcet disease (BD), and to discuss lesser-known features of pulmonary BD such as clinical characteristics, analysis of prognosis, and evaluation of treatment options with respect to the previously published cases. DESIGN: We conducted a comprehensive review of the literature to analyze cumulated data about pulmonary involvement in BD. SETTING: We found 159 articles regarding pulmonary disease associated with BD in May 2003. PATIENTS: The evaluation of these articles demonstrated 598 pulmonary problems in 585 cases. RESULTS: Pulmonary artery aneurysms (PAAs) are the most common pulmonary lesion in BD, and these are almost always associated with hemoptysis. Seventy-eight percent of patients with aneurysms have concomitant extrapulmonary venous thrombi or thrombophlebitis. Other pulmonary problems are reported in BD, and these are principally related to vascular lesions and radiologic abnormalities. CONCLUSIONS: Pulmonary vascular problems, either PAA or involvement of small-sized vessels, are the main pulmonary disorders in BD. Immunopathologic findings indicate that the underlying pathogenesis is pulmonary vasculitis, which may result in thrombosis, infarction, hemorrhage, and PAA formation. Patients with small nonspecific radiologic abnormalities should be followed up closely since early diagnosis of vascular lesions may be life-saving. Immunosuppression is the main therapy for the treatment of a vasculitis. It is important that pulmonary angiitis is not mistaken for pulmonary thromboembolic disease since fatalities have occurred in BD shortly after initiation of anticoagulation/thrombolytic treatment.

  1. Inspiratory and expiratory HRCT findings in Behcet's disease and correlation with pulmonary function tests. Ozer C, Duce MN, Ulubas B, Bicer A, Tursen U, Apaydin FD, Yildiz A, Camdeviren H. Eur J Radiol. 2005 Oct;56(1):43-7.
    PURPOSE: The purpose of our study was to describe the pulmonary parenchymal changes of Behcet's disease using high-resolution computed tomography and to correlate them with pulmonary function tests. MATERIALS AND METHODS: Thirty-four patients with Behcet's disease (18 men, 16 women), 3 of whom were symptomatic, were included as the study group. Four of 34 patients were smokers. Twenty asymptomatic volunteers (12 men, 8 women), 4 of whom were smokers, constituted the control group. The pulmonary function tests and high-resolution computed tomography were performed for both groups. RESULTS: Inspiratory high-resolution computed tomography findings were abnormal in nine patients (26.5%) of the study group. In eight patients, there were multiple abnormalities, whereas one patient had only one abnormality. Pleural thickening and irregularities, major fissure thickening, emphysematous changes, bronchiectasis, parenchymal bands, and irregular densities, and parenchymal nodules were the encountered abnormalities. Inspiratory high-resolution computed tomography scans were normal in the control group. On expiratory scans, there was statistically significant difference between study group and control group when air trapping, especially grades 3 and 4, was compared (P<0.01). Pulmonary function tests of both the study and the control groups were in normal ranges, and there was no statistically significant difference between the two groups according to pulmonary function tests (P>0.05). DISCUSSION AND CONCLUSION: High-resolution computed tomography is sensitive in the demonstration of pulmonary changes in patients with Behcet's disease. End-expiratory high-resolution computed tomography examination is very useful and necessary to show the presence of air trapping, thus the presence of small airway disease, even if the patient is asymptomatic or has normal pulmonary function tests.

  1. Audio-vestibular disturbances in Behcet's patients: report of 62 cases. Kulahli I, Balci K, Koseoglu E, Yuce I, Cagli S, Senturk M. Hear Res. 2005 May;203(1-2):28-31.
    This study was carried out to determine the characteristics and incidence of hearing loss and vestibular disturbance in Behcet's syndrome with a large number of patients. Sixty-two patients with Behcet's syndrome were included in this study, 34 men and 28 women whose mean age was 33.7 (15-60). Sixty-two healthy normal control subjects (38 male and 24 female) were included. Patient and control groups were questioned about any history of audio-vestibular disturbance and underwent physical and ENT examination and the following audiologic tests: pure tone audiometric test (0.25, 0.5, 1, 2, 4, and 6 kHz), tympanogram, speech discrimination, short increment sensitivity index, tone-decay test, auditory brainstem response. Vestibular system was evaluated by videonistagmogram and caloric test. Cranial and brainstem magnetic resonance imagine (MRI) of patients who have vestibular disturbances were practiced to examine the central nervous system. Both the patient and the control groups were tested with the HLA-B51 antigen. Pure tone audiogram showed sensory-neural hearing loss (25 dB hearing level in at least two frequencies) in 20 of the 62 (32%) Behcet's patients while the control group were normal. There was a hearing loss involving high frequencies in the audiograms of Behcet's patients with hearing disturbances. The recruitment investigation tests and auditory brain stem response confirmed cochlear involvement in all 20 patients. Caloric stimulation tests revealed a normal vestibular function in all patient and control group. In electronystagmography, 21 (34%) patients had hypometric or hypermetric saccades and smooth pursuit tests showing that 4 (6%) patients had pathological changes while the control group was normal. HLA-B51 antigen was found positive in 15 of 20 Behcet's patient with hearing loss. Conclusion: (1) The hearing and vestibular disturbances in Behcet's syndrome is more prevalent than previously recognized; (2) Hearing loss in high frequencies in Behcet's patients is an indicator of cochlear involvement in this disease; (3) There is a higher prevalence of central vestibular syndrome in Behcet's patients than it was thought before; (4) HLA-B51 antigen may be able to be a prognostic factor for sensorineural hearing loss in Behcet's patients.

  1. Clinical evaluation of Behcet's disease: a five year follow-up study. Yucel A, Sonmezoglu Marakli S, Aksungur VL, Uzun S, Sertdemir Y, Alpsoy E. J Dermatol. 2005 May;32(5):365-70.
    The frequencies of clinical features of Behcet's disease vary in different studies according to the diagnostic criteria used in the study, the department where the study is done, and the ethnic origin of patients included in the study. In this study, we aimed to evaluate the influence of the length of the follow-up period of patients on the frequencies of clinical features of Behcet's disease. Out of 231 patients who applied to our dermatology clinic from June of 1991 to October of 1996, only 52 patients that were followed up for at least five years were included in this retrospective study. The baseline characteristics of these 52 patients were almost the same as those of the 179 patients who dropped out. Almost all of the frequencies of clinical features at the end of the follow-up period of five years were higher than those at the time of presentation. The most prominent increases were observed for erythema nodosum (59.6% to 75.0%), papulopustular lesions (84.6% to 94.2%), and large vessel involvement (23.1% to 30.8%). According to our results, it is most likely that studies mainly including mostly patients with long-standing disease will report higher figures for the frequencies of these clinical features of Behcet's disease.

  1. Sensitivity and specificity of different diagnostic criteria for Behcet's disease in a group of Turkish patients. Dervis E, Geyik N. J Dermatol. 2005 Apr;32(4):266-72.
    Because there are no diagnostic laboratory tests or histopathologic findings, the diagnosis of Behcet's disease (BD) is based on clinical criteria. Various diagnostic criteria have been proposed. In this study, we assessed the sensitivity, specificity and the relative value of the Mason & Barnes, Japanese, Hamza, O'Duffy, Cheng & Zhang, Dilsen and International Study Group (ISG) criteria. Two hundred twenty-eight patients with Behcet's disease diagnosed according to either O'Duffy's or ISG criteria and 89 control patients with a variety of connective tissue diseases, including sarcoidosis, uveitis, erythema nodosum, and granulomatous intestinal disease, were evaluated. ISG and Hamza criteria showed the highest specificity (100%) in discriminating control subjects from the entire cohort. The highest sensitivity value (100%) was found in the Cheng & Zang criteria, which were found to be the most sensitive but the least specific (74.2%). The Hamza criteria had the highest relative value (sensitivity plus specificity) (198.2). Dilsen (196.9) and ISG (196.5) criteria also had high relative values. In this study, Hamza, Dilsen, and ISG criteria performed quite well in diagnosing BD in our country compared to the other criteria sets.

  1. Behcet's disease in Lebanon: clinical profile, severity and two-decade comparison. Hamdan A, Mansour W, Uthman I, Masri AF, Nasr F, Arayssi T. Clin Rheumatol. 2006 May;25(3):364-7. Epub 2005 Nov 15.
    The aim of this study is to describe the clinical features of Lebanese patients with Behcet's disease (BD) followed up at a tertiary care center in Lebanon. A retrospective review of medical records of 90 patients who fulfilled the International Study Group (ISG) criteria for diagnosis was performed. The clinical characteristics and severity score were compared with those reported from other populations of Arab and of Turkish origin using the same diagnostic criteria, and a comparison over two decades for the Lebanese population was made. The male-to-female ratio was 2.9:1, the mean age at onset, 25.4 years, and mean age at diagnosis, 29.2 years. One hundred percent of the patients had mouth ulceration; 72.2%, genital ulceration; 59.1%, arthritis; 55.7%, papulopustular skin lesion; 53.9%, ocular disease; 36.8%, vascular disease; 29.5%, erythema nodosum; and 23.0%, neurologic disease. The characteristics of our patient population are similar to that reported from other countries in the region. However, we found a higher prevalence of vascular and neurologic disease.

  1. Clinical features of Behcet's disease in children. Borlu M, Uksal U, Ferahbas A, Evereklioglu C. Int J Dermatol. 2006 Jun;45(6):713-6.
    BACKGROUND: Behcet's disease (BD) is a systemic inflammatory vasculitis of young adults with unknown etiology. OBJECTIVE: The aim of this study was to determine the clinical features and outcome of BD in childhood. PATIENTS AND METHODS: Seventeen patients with BD within a pediatric age group were studied since 1997. RESULTS: Boys : girls ratio was 12:5. Familial cases were very frequent (45%). Oral aphthous ulcers were present in all patients (100%), genital aphthous ulcers were present in 16 (94%), ocular lesions in 4 (24%), erythema nodosum in 3 (18%), papulopustules in 8 (47%), joint symptoms in 13 (76%), mild gastrointestinal symptoms in 2 (12%), neurological involvement in 2 (12%), positive pathergy reactions in 13 (76%), and thrombophlebitis in 1 (6%). We used colchicine, systemic corticosteroids, cyclosporine, nonsteroidal anti-inflammatory drugs, sulfasalazine, and topical agents for the treatment of children with BD. CONCLUSION: The findings of BD in children are similar as in adults, but the frequency of familial cases is significantly higher.

  1. The proportional Venn diagram of Behcet's disease-related manifestations among young adult men in Turkey. Dinc A, Bayir A, Simsek I, Erdem H, Pay S, Turan M. Clin Exp Rheumatol. 2005 Jul-Aug;23(4 Suppl 38):S86-90.
    OBJECTIVES: To determine the frequency of the features associated with Behcet's disease (BD) in a young men population and generate a proportional Venn diagram of those features. METHODS: Data was collected from 3714 otherwise healthy men recruited for military service at the entrance. Study was conducted in a two-step procedure. Firstly, all participants were questioned by a general practitioner via using visual Behcet's questionnaire. Those participants, in whom at least one BD-related manifestation of the disease (oral ulcer, genital ulcer, folliculitis, erythema nodosum, uveitis, venous involvement of the lower extremities) have been demonstrated, were further examined by a rheumatologist at the second-stage of the study. RESULTS: The areas of intersection among the 6 individual BD-related manifestations produced 63 mutually exclusive symptom groups. Sixteen out 63 of these groups were functionally operative in our study population. Forty-seven (1.2%) of the all participants were considered to have at least 1 of the BD-related manifestation after examined by rheumatologist. The prevalence rates of the individual manifestations among the study population were as follows; oral ulcer 29 (0.78%), folliculitis 31 (0.83%), genital ulcer 9 (0.24%), venous involvement 13 (0.35%), erythema nodosum 4 (0.10%) and uveitis 3 (0.08%). The group consisting of oral ulcer with folliculitis was the largest proportion of participants followed by the group having oral ulcer only, accounting for 0.29% and 0.18%, respectively. Four (0.1%) of the participants were fulfilled the International Study Group for BD criteria following rheumatologic and ophthalmologic examinations. After excluding the group having oral ulcer with folliculitis, additional 12 cases had features suggesting BD though they didn't fulfill the International Study Group for BD criteria. CONCLUSION: The Venn diagram of this study demonstrates that International Study Group for BD criteria can detect almost the quarter of 16 cases suspected as having BD. We suggest that the application of information regarding the frequencies of individual BD-related manifestations and their association with each other in a general population might serve as a helpful tool for physicians while making diagnosis.

  1. Behcet's disease and pregnancy. Jadaon J, Shushan A, Ezra Y, Sela HY, Ozcan C, Rojansky N. Acta Obstet Gynecol Scand. 2005 Oct;84(10):939-44. (Review)
    BACKGROUND: Behcet's disease (BD) is a multisystem inflammatory chronic disorder, which is characterized by relapsing oral and genital ulceration and iridocyclitis. While being of unknown etiology, vasculitic changes of possible autoimmune origin are common to all involved organs, and thrombotic complications, which may adversely affect gestation, are frequently seen. Very little is known to date about the reciprocal influence of BD and pregnancy. We have undertaken to explore the mutual effect of BD and pregnancy with emphasis on maternal and fetal complications. METHODS: In this case-control study, we have evaluated pregnancies that occurred in women suffering from BD, who were treated in our institution during the last 25 years. All records were reviewed, and data were confirmed by a telephone interview and compared with a matched control group. To review the current knowledge, a Medline search together with a manual search of selected articles was performed. RESULTS: Thirty-one Behcet's patients who had 135 pregnancies were studied. Remissions were significantly more frequent during both pregnancy and postpartum periods, while exacerbations were observed only in one-sixth of the patients (P < 0.001). Pregnancy complications (P < 0.001), cesarean section (P < 0.001), and miscarriage (P < 0.02) rates, however, were significantly higher in the study group. CONCLUSIONS: Our study suggests that pregnancy does not have a deleterious effect on the course of BD and may possibly ameliorate its course. However, it seems that BD may adversely affect pregnancy. The miscarriage rate was higher, and the pregnancy complications and cesarean section rates were significantly elevated.

  1. New approaches in the treatment of Adamantiades-Behcet's disease. Pipitone N, Olivieri I, Cantini F, Triolo G, Salvarani C. Curr Opin Rheumatol. 2006 Jan;18(1):3-9. (Review)
    PURPOSE OF REVIEW: To update clinicians on the recent advances in the treatment of Adamantiades-Behcet's disease. RECENT FINDINGS: Interferon-alpha-2a and infliximab have proved able to induce prompt remission in the vast majority of Adamantiades-Behcet's patients with DMARD-resistant uveoretinitis. Efficacy of interferon-alpha-2a has also been reported for mucocutaneous lesions, arthritis, and (more anecdotally) for neuro-Behcet, while results from small case series suggest that infliximab is beneficial for mucocutaneous lesions and (more anecdotally) for arthritis and gastro-intestinal manifestations. Two cases of neuro-Behcet treated with infliximab showed a complete resolution. Finally, in a randomized controlled trial of patients with mucocutaneous, arthritic manifestations, or both, etanercept effectively suppressed mucocutaneous lesions.A different approach is tolerization by oral administration of the 336-351 peptide of the human heat shock protein 60 (thought to have a pathogenic role in Adamantiades-Behcet's disease-associated uveitis), linked to recombinant cholera B-toxin B-subunit. Preliminary results have shown that tolerization is safe and effective in preventing relapses of uveitis. SUMMARY: Biologic agents have proved effective in patients resistant to conventional treatment. However, disease subsets characterized by severe morbidity and mortality such as vasculo-Behcet and neuro-Behcet still pose major therapeutic challenges. Further studies are needed to devise better treatment strategies for severe Adamantiades-Behcet's disease.

  1. Current concepts in the etiology and treatment of Behcet disease. Evereklioglu C. Surv Ophthalmol. 2005 Jul-Aug;50(4):297-350. (Review)
    Behcet disease is an idiopathic, multisystem, chronic, and recurrent disease characterized by exacerbations alternating with phases of quiescence, episodic panuveitis, and aggressive non-granulomatous occlusive vasculitis of the arteries and veins of any size with explosive ocular inflammatory attacks that primarily affect the retinal and anterior segment vasculature of the eye. The disease is characterized by endothelial dysfunction and may produce a wide variety of symptoms. In mild cases, orogenital ulcers and skin lesions are the only findings during the entire clinical course, whereas ocular lesions that occur in more than half of the cases, frequently bilateral, can eventually lead to blindness. Pulmonary, gastrointestinal, and central nervous system involvements may sometimes be life-threatening. This review examines the epidemiology, frequency, immunology, and immunohistopathology of Behcet disease with recent theories of several agents, including phosphoantigens, superantigens, heat-shock proteins, and adenosine deaminase. Perspectives on the possible roles of new etiopathological molecules, such as nitric oxide, endothelin, and homocysteine, are presented. Ocular and systemic clinical features, diagnostic criteria, classifications, laboratory, fundus fluorescein angiography, and radiologic imaging are discussed. Differential diagnosis, disease in pregnancy and childhood, and prognosis with regard to Behcet-induced systemic and ocular complications are also evaluated. Traditional and current treatments with topical, paraocular and systemic corticosteroids, colchicine, dapsone, cyclosporine, azathioprine, methotrexate, cyclophosphamide and chlorambucil are summarized and recent insights into the pharmacology and effects of thalidomide, tacrolimus (FK-506), interferon-alpha, anti-TNF-alpha blocking monoclonal autoantibody (infliximab) and soluble TNF receptor (etanercept) are reviewed. Key clinical investigations with the status of ongoing clinical trials aimed at addressing the drug's efficacy, surgical care, and studies that have raised the possibility of new therapeutic uses are also presented. The challenges posed by the drug's teratogenicity and adverse effects are also considered, if present.

  1. Number VII Behcet's disease (Adamantiades syndrome). Escudier M, Bagan J, Scully C. Oral Dis. 2006 Mar;12(2):78-84. (Review)
    Behcet's syndrome (BS; Adamantiades syndrome) is the association of the triple symptom complex of recurrent aphthous stomatitis (RAS) with genital ulceration, and eye disease (especially iridocyclitis) though a number of other systemic manifestations may also be seen. BS mainly affects young adult males, and there is an association with HLA-B5 and HLA-B51 (B5101). Features such as arthralgia and leucocytoclastic vasculitis suggest an immune-complex mediated basis, which is supported by finding circulating immune complexes and, although the antigen responsible is unidentified, heat shock proteins have been implicated. An inflammatory disorder, BS is now considered as a systemic vasculitis, characterised by a very wide spectrum of clinical features and by unpredictable exacerbations and remissions.

  1. Behcet's disease. Bonfioli AA, Orefice F. Semin Ophthalmol. 2005 Jul-Sep;20(3):199-206. (Review)
    Behcet's disease is a chronic, relapsing vasculitis that can affect most organ systems. The prevalence varies geographically, and the disease is more common in countries along the ancient Silk Road, including Italy, Turkey, Israel, Saudi Arabia, Iran, China, Korea and Japan. Behcet's is more common in men than in women, and typically affects young adults. The classic finding in Behcet's patients is the presence of recurrent mucocutaneous ulcers, and oral aphthous ulcerations are usually the initial symptom. Other manifestations include genital ulcers, skin lesions, vascular, neurological, articular, and ocular disease. The disease can affect the anterior and/or posterior segments of the eye, and the main manifestations include iridocyclitis, hypopyon, mild to moderate vitreitis, retinal vasculitis and occlusion, optic disc hyperemia, and macular edema. There is no pathognomonic laboratory test in Behcet's disease, and the diagnosis is based in systemic and ocular clinical findings. Treatment of ocular Behcet is based in corticosteroids and immunosuppressive agents, to suppress acute inflammation and reduce its recurrence frequency. Ocular lesions may improve with immunosuppressive therapy, but usually are not fully reversible, and generally progress over time. The prognosis of anterior uveitis is usually good, but patients with posterior lesions tend to have some degree of visual loss, even with adequate treatment.

  1. Behcet's disease: familial clustering and immunogenetics. Fietta P. Clin Exp Rheumatol. 2005 Jul-Aug;23(4 Suppl 38):S96-105. (Review)
    Behcet's disease (BD) is a relapsing, multisystemic inflammatory disorder, characterized by major symptoms consisting of recurrent orogenital ulcerations, eye and skin lesions. Other clinical features may include musculoskeletal, vascular, gastrointestinal, renal, cardiopulmonary or neurological involvement. Vasculitis affecting all types and sizes of blood vessels is the main histopathologic process, in a third of cases complicated by thrombosis. The etiopathogenesis is presently unknown, but BD likely represents the result of a peculiar immune response to hitherto unidentified environmental factors in genetically predisposed subjects. The prevalent distribution in a specific geographical area spanning the Mediterranean basin and Asia, the close association with human leukocyte antigen B*51 in different ethnic groups, and the familial clustering of BD are hallmarks accounting for the strong contribution of a genetic background. The BD familial aggregation is characterized by both genetic anticipation and higher prevalence in childhood patients, likely defining a subset with stronger immunogenetic influences. Polymorphisms in genes encoding for host effector molecules may have a supplementary role in disease susceptibility and/or severity. The contribution of prothrombotic mutations and polymorphisms in the pathogenesis of BD thrombosis is controversial. In this paper, the available reports on BD familial clustering and the evidence for the role of immunogenetic predisposing factors are reviewed.

  1. Orogenital ulceration with overlapping tuberculosis: epiphenomenon or expanding spectrum of Behcet disease? Hamill M, Remedios D, Kapembwa M. J Low Genit Tract Dis. 2006 Oct;10(4):219-22.
  2. Intestinal perforation due to miliary tuberculosis in a patient with Behcet's disease. Kapan M, Karabicak I, Aydogan F, Kusaslan R, Kisacik B. Mt Sinai J Med. 2006 Sep;73(5):825-7.
  3. Vascular Behcet's disease with coronary artery aneurysm. Arishiro K, Nariyama J, Hoshiga M, Nakagawa A, Okabe T, Nakakoji T, Negoro N, Ishihara T, Hanafusa T. Intern Med. 2006;45(15):903-7. Epub 2006 Sep 1.
  4. Fever of unknown origin as the initial presenting sign of Behcet's disease. Erkek E, Ayaslioglu E. Scand J Infect Dis. 2006;38(9):829-30.
  5. A Behcet's disease patient with intracardiac thrombus, pulmonary artery aneurysms complicating recurrent pulmonary thromboembolism. Ernam D, Atalay F, Alp A, Hasanoglu HC. Tuberk Toraks. 2006;54(2):168-71.
  6. Dementia in a case of Behcet's disease. Warren JE, Sandhu A, Thompson PD, Blumbergs PC. Intern Med J. 2006 Sep;36(9):611-6.
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  10. Transverse aortic arch replacement associated with MAGIC syndrome: case report and literature review. Caceres M, Estrera AL, Buja LM, Safi HJ. Ann Vasc Surg. 2006 May;20(3):395-8. Epub 2006 Apr 26.
  11. Behcet's disease: a rare cause of vulval ulcer in a perimenopausal woman. Gowri V, Nair P. Acta Obstet Gynecol Scand. 2006;85(6):760-1.
  12. Pseudoaneurysm of the internal iliac artery of a patient with Behcet's disease: treatment with an endovascular stent-graft. Tola M, Yurdakul M, Ozdemir E, Ozbulbul N, Koktekir E, Cumhur T. Cardiovasc Intervent Radiol. 2006 Jul-Aug;29(4):720-2.
  13. Possible isotretinoin-induced keloids in a patient with Behcet's disease. Dogan G. Clin Exp Dermatol. 2006 Jul;31(4):535-7.
  14. Encapsulated triamcinolone cyst after subtenon injection. Chan CK, Mohamed S, Tang EW, Shanmugam MP, Chan NR, Lam DS. Clin Experiment Ophthalmol. 2006 May-Jun;34(4):360-2.
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  16. An unusual cause of a cerebral tumour in a young patient. Behcet's disease. Kosters K, Bos MM, Wesseling P, Smeets SM, van der Ven AJ, Bredie SJ. Neth J Med. 2006 May;64(5):152, 163.
  17. Intestinal Behcet's disease with esophageal ulcers and colonic longitudinal ulcers. Fujiwara S, Shimizu I, Ishikawa M, Uehara K, Yamamoto H, Okazaki M, Horie T, Iuchi A, Ito S. World J Gastroenterol. 2006 Apr 28;12(16):2622-4.
  18. An unusual case of neuro-Behcet's disease presenting with reversible basilar artery occlusion. Lee H, Yi HA, Lee SR, Nam SI, Lee J. Neurol Sci. 2006 Apr;27(1):70-3.
  19. Myositis in Behcet's disease after tonsillectomy. Abadi U, Hadary R, Shilo L, Shabun A, Greenberg G, Kovatz S. Isr Med Assoc J. 2006 Apr;8(4):294-5.
  20. A case of Behcet's disease with pericarditis, thrombotic thrombocytopenic purpura, deep vein thrombosis and coronary artery pseudo aneurysm. Kwon CM, Lee SH, Kim JH, Lee KH, Kim HD, Hong YH, Lee CK. Korean J Intern Med. 2006 Mar;21(1):50-6.
  21. Behcet's aortitis mimicking aortic valve endocarditis with subaortic complications. Shiran A, Zisman D, Karkabi B, Safadi T, Aravot D, Bitterman H, Lewis BS. J Am Soc Echocardiogr. 2006 May;19(5):578.e1-4.
  22. Epilepsia partialis continua in a patient with Behcet's disease. Aktekin B, Dogan EA, Oguz Y, Karaali K. Clin Neurol Neurosurg. 2006 Jun;108(4):392-5.
  23. Allogeneic bone marrow transplantation (BMT) for refractory Behcet's disease with severe CNS involvement. Marmont AM, Gualandi F, Piaggio G, Podesta M, Teresa van Lint M, Bacigalupo A, Nobili F. Bone Marrow Transplant. 2006 Jun;37(11):1061-3.
  24. Orbital inflammation associated with Behcet's disease. Hammami S, Yahia SB, Mahjoub S, Khairallah M. Clin Experiment Ophthalmol. 2006 Mar;34(2):188-90.
  25. A case of primary biliary cirrhosis complicated by Behcet's disease and palmoplantar pustulosis. Iwadate H, Ohira H, Saito H, Takahashi A, Rai T, Takiguchi J, Sasajima T, Kobayashi H, Watanabe H, Sato Y. World J Gastroenterol. 2006 Apr 7;12(13):2136-8.
  26. Macular star associated with Behcet disease. Chan RV, Lee TC, Chaganti RK, Cestari DM, Kim MT, Lee S. Retina. 2006 Apr;26(4):468-70.
  27. Rapidly reversible neurological manifestations in a case of neuro-Behcet disease. Ishii M, Shiraiwa N, Komatsu T, Kashiwagi H. Intern Med. 2006;45(5):343-4. Epub 2006 Apr 3.
  28. Neuro-Behcet disease presenting as secondary pseudotumor syndrome: case report. Can E, Kara B, Somer A, Keser M, Salman N, Yalcin I. Eur J Paediatr Neurol. 2006 Mar;10(2):97-9. Epub 2006 Apr 3.
  29. Endovascular repair for a huge vertebral artery pseudoaneurysm caused by Behcet's disease. Dong ZH, Fu WG, Guo DQ, Xu X, Chen B, Jiang JH, Yang J, Shi ZY, Wang YQ. Chin Med J (Engl). 2006 Mar 5;119(5):435-7.
  30. Hepatic artery aneurysm presenting with hemobilia in a patient with Behcet's disease: treatment with percutaneous transcatheteral embolization. Hatzidakis A, Petrakis J, Krokidis M, Tsetis D, Gourtsoyiannis N. Diagn Interv Radiol. 2006 Mar;12(1):53-5.
  31. A giant aneurysm of the left main coronary artery in a patient with Behcet's disease. Dogan SM, Aydin M, Gursurer M, Onuk T. Tex Heart Inst J. 2006;33(2):269.
  32. Intestinal Behcet's disease with pyoderma gangrenosum: a case report. Nakamura T, Yagi H, Kurachi K, Suzuki S, Konno H. World J Gastroenterol. 2006 Feb 14;12(6):979-81.
  33. Extracranial carotid aneurysm in Behcet disease: Report of two new cases. Bouarhroum A, Sedki N, Bouziane Z, El Mahi O, El Idrissi R, Lahlou Z, Lekehel B, Sefiani Y, El Mesnaoui A, Benjelloun A, Ammar F, Bensaid Y. J Vasc Surg. 2006 Mar;43(3):627-30.
  34. Efficacy of TNF {alpha} blockade in cyclophosphamide resistant neuro-Behcet disease. Ribi C, Sztajzel R, Delavelle J, Chizzolini C. J Neurol Neurosurg Psychiatry. 2005 Dec;76(12):1733-5.
  35. Severe mucocutaneous Behcet's disease treated with dexamethasone pulse. Verma KK, Tejasvi T, Verma K, Sethuraman G, Bansal A. J Assoc Physicians India. 2005 Nov;53:998-9.
  36. Behcet disease with an intrapulmonary shunt. Tuncel M, Kiratli PO, Mahmoudian B. Clin Nucl Med. 2006 Mar;31(3):181-2.
  37. A case of polychondritis in a patient with Behcet's disease. Kim MK, Park KS, Min JK, Cho CS, Kim HY. Korean J Intern Med. 2005 Dec;20(4):339-42.
  38. Aseptic meningitis, venous sinus thrombosis, intracranial hypertension and callosal involvement contemporaneously in a young patient with Behcet's disease. Bir LS, Sabir N, Kilincer A, Celiker A, Erdogan BS. Swiss Med Wkly. 2005 Nov 19;135(45-46):684.
  39. Rhinosinusitis in a patient with Behcet's syndrome. Alcantara LJ, Trentin Junior A, Mocellin M, Mira JG, Robert F, Rosa OA. Rev Bras Otorrinolaringol (Engl Ed). 2005 Mar-Apr;71(2):250-3. Epub 2005 Aug 2.
  40. Myocardial infarction and deep venous thrombosis in a young patient with Behcet disease. Calguneri M, Aydemir K, Ozturk MA, Haznedaroglu IC, Kiraz S, Ertenli I. Clin Appl Thromb Hemost. 2006 Jan;12(1):105-9.
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  42. Pregnancy complicated by superior vena cava thrombosis and pulmonary embolism in a patient with Behcet disease and the use of heparin for treatment. Kale A, Akyildiz L, Akdeniz N, Kale E. Saudi Med J. 2006 Jan;27(1):95-7.
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  80. Heterotopic ossification related to toxic epidermal necrolysis in a patient with Behcet's disease. Samanci N, Balci N, Alpsoy E. J Dermatol. 2005 Jun;32(6):469-73.
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  1. Is the long-term use of systemic corticosteroids beneficial in the management of Behcet's syndrome? Hirohata S. Nat Clin Pract Rheumatol. 2006 Jul;2(7):358-9.
  2. Behcet's disease and familial Mediterranean fever. Takeno M, Ishigatsubo Y. Intern Med. 2006;45(13):805-6. Epub 2006 Aug 1. (Editorial)
  3. Behcet's disease and other autoinflammatory conditions: what's in a name? Yazici H, Fresko I. Clin Exp Rheumatol. 2005 Jul-Aug;23(4 Suppl 38):S1-2. (Editorial)
  4. Clinical features of Japanese patients with Behcet's disease and MICA polymorphism. Nishiyama M, Takahashi M, Manaka K, Suzuki S, Saito M, Nakae K. Jpn J Ophthalmol. 2005 Sep-Oct;49(5):424-6. (Letter)
  5. Behcet's disease in Poland. Kotulska A, Kucharz EJ, Kopec M. Clin Rheumatol. 2005 Nov;24(6):667-8. Epub 2005 Apr 29. (Letter)
  6. Impaired coronary microvascular functions in patients with Behcet disease. Gullu H, Caliskan M, Erdogan D, Yilmaz S, Dursun R, Ciftci O, Yucel E, Muderrisoglu H. J Am Coll Cardiol. 2006 Aug 1;48(3):586-7. Epub 2006 Jul 12. (Letter)

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