Listed during August 2004 – April 2005

Ophthalmology is a critical discipline in the management of Behçet's disease (BD). Therefore we askedDr. M. Zierhut from the Department of Ophthalmology, University of Tuebingen (Germany) and thank him for his review on the most recent papers on Behçet's eye manifestations for us:

“BD often leads to severe ocular involvement, so every year several papers expand our information about this severe problem. In the last 6 months, 6 papers reported on various aspects of this complication:

Tugal-Tutkun et al. reported about 880 BD-uveitis patients from Turkey, analysed between 1980 and 1998. They found 68% males, with a mean age of onset of uveitis of 28.5 years (males) and 30 years (females). Panuveitis was the most common form, and males showed more severe disease than women. As has been shown also in studies from other countries, the prognosis in the 90´s has been improved compared to the 80´s.

Besides uveitis, acute optic neuropathy can be a rare ocular complication, as has been reported in 1 patient by Yalcindag et al. After mega-dose steroid treatment the outcome there was complete resolution of the optic nerve swelling.

One of the most severe ocular inflammations is the so called “acute retinal necrosis syndrome” (ARN), nearly always initiated by viruses of the Herpes group. In their work Balansard et al. analysed 16 patients with the clinical impression of ARN and found 1 patient with BD. This demonstrates that ocular BD may look very similar to ARN.

One of the complications of ocular BD, like all intraocular inflammations, is the development of glaucoma. Due to elevated intraocular pressure, glaucoma leads to a degeneration of the optic nerve, ending up in blindness. The glaucoma develops due to inflammation or treatment (steroids) or as a complication of changes of the anterior anatomy of the eye (cataract, synechia formation, angle closure). Elgin et al. analysed 230 eyes from 129 patients regarding a possible glaucoma. Secondary (induced by inflammation) glaucoma was found in 10.9 %. Of these patients 44% had steroid or inflammation induced glaucoma, 24% partial angle-closure glaucoma, and 12 % pupil block (intraocular fluid can not reach the draining vessels, leads to acute glaucoma). Treatment with drugs was mostly ineffective, as it is typical for secondary glaucoma. Various surgical procedures had been started, but the final success is not reported.

In angle open glaucoma, a procedure called “trabeculectomy” can be performed. Here, a channel between anterior chamber and the conjunctiva is installed, leading to a filtering bleb. This is a nice procedure when this bleb remains open. Unfortunately, especially in secondary glaucomas, this artificial hole often heals, and the success slows down to zero. In these “high-risk patients” the topical treatment with the cytostatic mitomycin-C has generally improved the prognosis. Yalvac et al. report about 26 patients, receiving this regimen. After a mean follow-up of 40 months, they found 23 % of the patients requiring no additional treatment. The visual acuity improved or remained within two lines of the preoperative visual acuity in 73.1%. Postoperatively 23.1% developed cataract, only 1 patient lost his eye due to phthisis bulbi (eye without pressure). They conclude that this procedure appears to provide long-term safety and effectiveness for uveitic glaucoma in BD eyes”

From the neurological perspective we found interesting that according to a subcommittee of the International Headache Society for headache classification (ICHD-II) secondary headaches may occur in patients affected by inflammatory diseases of the central nervous system (CNS), and headache and/or primary headache is reported in 55% of BD patients (La Mantia et al.). In neuro-Behcet's disease discrepancies between MRI and Tc-99m HMPAO SPECT findings were described by Cengiz et al. (study size 12 patients). Brain SPECT may act as a complementary modality to increase the detection rate of affected regions in patients with neuro-BD.

TNF-alpha blocking agents are important for the treatment of severe BD, especially in case of eye manifestations. In the last months several cases have been reported for the treatment of refractory uveitis (n=4), recalcitrant idiopathic orbital inflammation (chronic orbital myositis, n=1), posterior uveitis with retinal neovascularization (n=1), and chronic cystoid macular edema associated with uveitis (n=2). All these cases responded to TNF-alpha blocking agents and thus support the importance of these agents in severe BD disease. (Wechsler, Garrity, Giansanti, Markomichelakis et al.) It is interesting to note, that etanercept, which was effective in suppressing most of the mucocutaneous manifestations of BD, did not affect the pathergy reaction and the cutaneous response to MSU crystals (Melikoglu et al).

In a Moroccan study, 50% of 12 pediatric BD patients had neuro-BD (Allali et al.)! During follow-up 4 patients with neurological involvement developed steroid-dependence and 4 patients had optic atrophy with blindness. Whether these children would profit from TNF-alpha blocking agents, remains open.

Although no abstract is provided, we would call the readers attention to a review of 26 pulmonary artery aneurysms diagnosed since 1992, from Istanbul (Hamuryudan et al.). Although the study is descriptive and uncontrolled, the mortality was considerably less than that of patients diagnosed earlier. Aggressive medical treatment with corticosteroids and cyclophosphamide, and, possibly, embolization for refractory hemoptysis may all be responsible for the improved survival of these patients. Large vessel disease in BD was reviewed in Current Opinion in Rheumatology in January 2005 (Calamia et al).

We also cite a case of tricuspid aseptic endocarditis in a patient with unrecognized BD, who was initially misdiagnosed and treated as infective endocarditis (Aouba et al.).

DETAILED CITATIONS AND ABSTRACTS (Case reports cited in small letters)

OPHTHALMOLOGY 3

NEUROLOGY 5

TNF-a BLOCKING AGENTS 6

MORE PAPERS 8

OPHTHALMOLOGY

Yalvac IS, Sungur G, Turhan E, Eksioglu U, Duman S. Ankara, Turkey.

J Glaucoma. 2004 Dec; 13 (6): 450 - 3

PURPOSE: To determine the effect of intraoperative application of Mitomycin-C (MMC) with trabeculectomy in uveitic glaucoma associated with Behcet disease. MATERIALS AND METHODS: Twenty-six eyes of 26 patients with uveitic glaucoma associated with Behcet disease who underwent trabeculectomy with MMC between 1996 and 2001 were reviewed in this retrospective, noncomparative study. Trabeculectomy + MMC in concentration of 0.4 mg/mL for 3 minutes was performed to all patients. Main outcome measures were control of IOP, the number of antiglaucoma medications required to achieve the desired IOP, visual acuity and complications. The surgical success was defined as IOP less than 22 mm Hg and greater than 5 mm Hg without additional further glaucoma surgery or loss of light perception. RESULTS: The cumulative probability of success was 83.3% at 1 year, 76.2% at 2 years, 70% at 3 years, 66.7% at 4 years and 62.5% at 5 years after surgery. The mean follow-up was 40.0+/-18.0 months. At last follow-up 23% of the patients required no antiglaucoma medications. Best-corrected visual acuity improved or remained within two lines of preoperative visual acuity in 19 eyes (73.1%). Glaucomatous (1 eye 3.8%) and nonglaucomatous optic atrophy (3 eyes 11.5%) was the most frequent reason for visual decrease (total 4 eyes 15.2%). The most common complications were cataract formation in 6 eyes (23.1%), bleb leakage in 4 eyes (15.3%) and choroidal effusion in 3 eyes (11.5%). Phthisis bulbi was found in one (3.8%) patient. CONCLUSION: Trabeculectomy and intraoperative application of MMC appears to provide long term safety and effectiveness in uveitic glaucoma associated with Behcet disease.

Elgin U, Berker N, Batman A. Ankara, Turkey.

J Glaucoma. 2004 Dec; 13 (6): 441 - 4

PURPOSE: To determine the incidence of secondary glaucoma in Behcet disease. METHODS: A total of 230 eyes of 129 patients with Behcet disease, were examined in uveitis and glaucoma clinics of Ankara Social Security Eye Hospital between January 1997 and September 2002. The data from all patients were investigated both retrospectively and prospectively. RESULTS: The mean age of 129 patients was 34.2 +/- 7.4 years (range, 18 to 55 years). In 22 patients (17%), the disease was diagnosed on the basis of the ocular findings, while in the remaining 107 patients (83%), the period between the diagnosis of Behcet disease and the onset of the ocular symptoms was 23.3 +/- 17 months (range, 1 month to 5.3 years); 122 eyes (53%) had the episodes of acute recurrent iridocyclitis, while 108 eyes (47%) developed chronic posterior uveitis, including vitreitis, retinitis, vasculitis, or optic nerve involvement. Secondary glaucoma was diagnosed in 25 eyes (10.9%); 11 eyes (44%) with steroid or inflammation induced open angle glaucoma, 6 eyes (24%) with partial angle-closure glaucoma and peripheral anterior synechiae, 5 eyes (20%) with angle closure glaucoma, peripheral anterior synechiae, and pupil block and 3 eyes (12%) with neovascular glaucoma. The treatments included YAG-laser iridotomy in 5 eyes, diode-laser cyclodestruction in 3 eyes, primary trabeculectomies with mitomycin-c in 4 eyes, secondary trabeculectomies with mitomycin-c in 2 eyes, Ahmed valve implantations in 2 eyes, and cyclocryotherapy in 3 eyes. CONCLUSIONS: We suggest that secondary glaucoma is a common and serious complication of Behcet disease. It develops as a result of multiple factors, generally triggered by recurrent intraocular inflammation. Early recognition and treatment of these factors have vital importance to avoid the visual morbidity.

Balansard B, Bodaghi B, Cassoux N, Fardeau C, Romand S, Rozenberg F, Rao NA, Lehoang P. Paris, France.

Br J Ophthalmol. 2005 Jan; 89 (1): 96 -101

AIM: To determine an aetiological diagnosis in patients presenting with necrotising retinopathies that simulate acute retinal necrosis (ARN). METHODS: Retrospective non-comparative case series. The charts of 16 patients presenting with a clinical impression of ARN at Pitie-Salpetriere Hospital, Paris, France, between 1994 and 1999, who required initial antiviral therapy were reviewed. All of the patients had extensive laboratory tests. Anterior chamber paracentesis was performed on 14 patients and evaluated by polymerase chain reaction (PCR) and/or the Witmer-Goldmann coefficient to determine the cause of retinitis. Three of the 14 cases also had diagnostic vitrectomy. Responses to specific treatment, initiated based on laboratory results, and the final outcome were evaluated. RESULTS: Seven of the 16 patients were female and nine were male. The retinitis was bilateral in five patients and unilateral in 11 patients. The average age of the patients at presentation was 53.6 years. 13 patients were immune deficient for various reasons. Upon initial presentation, the patients' visual acuities were less than 20/200 in 68% of the affected eyes. The final diagnoses, based on laboratory data and therapeutic response were toxoplasmic retinochoroiditis (62.5%), syphilitic retinitis (12.5%), aspergillus endophthalmitis (12.5%), Behcet's disease (6.2%), and intraocular lymphoma (6.2%). Visual acuity was stabilised or improved in 12 patients (75%). Two patients with aspergillosis died despite antifungal therapy. CONCLUSIONS: Toxoplasmic retinochoroiditis is the major cause of retinal necrosis that simulates ARN, and PCR analysis of the aqueous humour is helpful in establishing the diagnosis. Such atypical toxoplasma retinochoroiditis may be associated with poor visual outcome.

Tugal-Tutkun I, Onal S, Altan-Yaycioglu R, Huseyin Altunbas H, Urgancioglu M. Capa Istanbul, Turkey.

Am J Ophthalmol. 2004 Sep; 138 (3): 373 - 80

PURPOSE: We aimed to describe the demographic and clinical features, ocular manifestations, complications, visual prognosis, and treatment in a large population of Turkish patients with Behcet uveitis. We also aimed to compare visual prognosis between male and female sex and between patients who presented before and after 1990. DESIGN: Observational case series. METHODS: A retrospective study of 880 consecutive patients (1,567 eyes) with Behcet uveitis seen at the Uveitis Service, Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, from 1980 to 1998. All patients met the classification criteria of the International Study Group for Behcet's Disease. Information on the patient's sex, age at onset of uveitis, ocular features, ocular complications, visual acuity, and systemic treatment was collected. RESULTS: Five hundred ninety-nine patients (68%) were male and 281 (32%) were female. The mean age at onset of uveitis was 28.5 years in male and 30 years in female patients. Ocular involvement was bilateral in 78.1% and unilateral in 21.9% of the patients. Panuveitis was the most common form in both sexes. Fundus lesions as well as sight-threatening complications were more common in males. At the beginning of the follow-up, potential visual acuity was 0.1 or less in 30.9% of eyes in males and 24.2% of eyes in females. Kaplan-Meier survival analysis estimated the risks of losing useful vision (>0.1) at 5 and 10 years for males and females as 21% vs 10% and 30% vs 17%, respectively. Male patients who presented in the 1990s had a significantly lower risk of losing vision compared with male patients who presented in the 1980s. CONCLUSION: Behcet uveitis starts frequently around the end of the third decade and has a male predominance. The disease is more severe and the risk of losing useful vision is higher in males than in females. However, this risk has been significantly reduced in the 1990s.

Yalcindag N, Yilmaz N, Tekeli O, Ozdemir O. Ankara - Turkey.

Eur J Ophthalmol. 2004 Nov - Dec;14 (6): 578 - 80.

PURPOSE: To report a patient with Behcet disease presenting with acute optic neuropathy. METHODS: A 47-year-old man was admitted to the authors' clinic owing to sudden visual loss in the left eye. Ophthalmologic and systemic examinations were performed. RESULTS: Visual acuity was 4/10 in the left eye. Fundus examination demonstrated swollen optic disc with blurred margins. There was relative afferent pupil defect in the same eye. Mega-dose corticosteroid treatment was started with the diagnosis of acute optic neuropathy. Ocular findings resolved within 2 weeks. CONCLUSIONS: Behcet disease rarely presents with acute optic neuropathy.

NEUROLOGY

Correlation of clinical, MRI and Tc-99m HMPAO SPECT findings in neuro-Behcet's disease.

Cengiz N, Sahin M, Onar M. Samsun, Turkey.

Acta Neurologica Belgica 2004 Sep; 104 (3): 100 - 5

Behcet's disease (BD) is a systemic disorder of unknown cause. In our study, we investigated the utility of Tc-99m HMPAO SPECT and MRI in patients with neuro-Behcet's disease (n-BD). PATIENTS AND METHODS: Twelve patients (two females, ten males; mean age 33 +/- 9.6 yr; age range 18-45 yr) with n-BD, fulfilling the criteria of the International Study Group for BD, were included in the study. MRI was performed according to a standard protocol with 1 or 1.5 T imagers. Brain SPECT data were obtained using a single head gamma camera after 555 MBq Tc-99m HMPAO injections. Following image reconstruction, regional cerebral perfusion was evaluated both visually and semi quantitatively. RESULTS: Neurological examination showed parenchymal brain involvement in 6 of 12 patients (50%). All 6 patients had white matter lesions on MRI, while only 5 of them showed perfusion defects on brain SPECT. Out of 6 patients (50%) without clinical signs of parenchymal brain involvement, 1 patient showed a pathological brain SPECT and 3 patients showed pathological MRI findings. Semiquantitative analysis of brain regions on SPECT study showed statistically significant hypoperfusion of biparietal regions. CONCLUSION: This study investigates the correlation between clinical, MRI and SPECT findings and it shows discrepancy between these findings in some cases. Brain SPECT may act as a complementary modality to increase the detection rate of affected regions in patients with n-BD.

La Mantia L, Erbetta A. Milan, Italy.

Neurological Sciences 2004 Oct; 25 Suppl 3: S148 - 53

The subcommittee of the International Headache Society for headache classification (ICHD-II) has recently recognised that secondary headaches may occur in patients affected by inflammatory diseases (ID) of the central nervous system (CNS), classifying them among the headaches attributed to non-vascular intracranial disorders. The aim of the study was to verify the association between headache and inflammatory non-infectious diseases of the CNS, by a review of the literature data on the topic, integrated by personal cases and data. Secondary headaches may occur in four main disorders: neurosarcoidosis (sec 7.3.1), aseptic (non-infectious) meningitis (7.3.2), other non-infectious ID (7.3.3) and lymphocytic hypophysitis (7.3.4). Headache and/or primary headaches are frequently reported in patients with neurosarcoidosis (30%), Behcet's syndrome (BS) (55%) and acute disseminated encephalomyelitis (45-58%). Recent data show a high incidence of headache also in multiple sclerosis (MS) (58%) (not mentioned in ICHD-II). The association between headache and inflammatory dysimmune diseases of the CNS, in particular BS and MS, might suggest a pathogenetic relationship.

Habituation of auditory event-related potentials in patients with Behcet's disease.

Kececi H, Kavak A, Akyol M, Degirmenci Y, Anul H. Duzce, Turkey.

Neurological Sciences 2004 Dec; 25 (5): 257 – 63

Behcet's disease is a progressive and/or relapsing-remitting multisystem inflammatory disorder. Involvement of the central nervous system occurs in 10%-29% of cases with Behcet's disease. The aim of the present study was to assess the effect of repetitive auditory stimulations on event-related potentials (ERP) in patients with Behcet's without neurologic manifestations. The study was performed in 14 patients with Behcet's disease and 14 healthy volunteers. ERPs were recorded from Fz, Cz and Pz. The auditory ERPs test was continued until 20 artefact-free rare tones were averaged, which was accepted as one trial block. After six blocks were obtained in a sequential manner, the test was completed. According to the results of the present study, P300 latency changed in different manners over trial blocks in the two groups. P300 latency values showed a progressive increase in the controls; however this was not determined in the Behcet's group. Thus, habituation was not observed in the patient group. Because of the lack of habituation related to P300 latency, it may be thought that there is an insidious pathologic process in Behcet's disease without neurologic manifestation.

Mirsattari SM, McGinn GJ, Halliday WC. Ontario, Canada.

Neurology 2004 Jul; 63 (2): 382 - 4

We report a patient with relapsing-remitting meningoencephalitis secondary to neuro-Behcet disease which resulted in recurrent brainstem encephalitis. MRI revealed increased signal intensity on the proton density and T2-weighted images and gadolinium enhancement during relapses. Autopsy revealed acute on chronic meningoencephalomyelitis involving the entire CNS, which was most marked in the brainstem.

Melikoglu M, Fresko I, Mat C, Ozyazgan Y, Gogus F, Yurdakul S, Hamuryudan V, Yazici H. Istanbul, Turkey.

J Rheumatol. 2005; 32: 98 -105

OBJECTIVE: To determine the effect of the tumor necrosis factor-alpha blocker etanercept on the pathergy and monosodium urate (MSU) status and on the mucocutaneous and articular manifestations of patients with Behcet's disease (BD). METHODS: Forty male patients with BD, all with positive pathergy and MSU tests and mucocutaneous disease and/or arthritis, were randomized (20 patients to each study arm) to receive either etanercept 25 mg twice a week or placebo for 4 weeks. The pathergy and MSU responses and the frequencies of mucocutaneous and articular manifestations were compared between the 2 groups. RESULTS: There were no decreases in the pathergy and MSU responses in the etanercept group compared to the placebo group at any time. The mean numbers of oral ulcers, nodular lesions, and papulopustular lesions were less in the etanercept group compared to the placebo group at all weekly evaluations, except for the second week for papulopustular lesions. The probability of being free of oral ulcers and nodular lesions was also significantly higher in the former group (log-rank chi-square = 9.83, p = 0.0017; log-rank chi-square = 14.17, p = 0.0002, respectively). CONCLUSION: Etanercept did not affect the pathergy reaction and the cutaneous response to MSU crystals. However, the drug was effective in suppressing most of the mucocutaneous manifestations of BD.

Infliximab in refractory uveitis due to Behcet's disease.

Wechsler B, Sable-Fourtassou R, Bodaghi B, Huong DL, Cassoux N, Badelon I, Fain O, LeHoang P, Piette JC. Paris, France.

Clin Exp Rheumatol 2004 Jul - Aug; 22(4 Suppl 34): S14 - 6

OBJECTIVE: To report 4 cases of refractory panuveitis due to Behcet's disease treated with a novel therapy: infliximab. METHODS: Retrospective study of 3 women and 1 man of Causasian origin with Behcet's disease complicated with panuveitis. Their uveitis was relapsing from 48 to 96 months and was resistant to the combination of colchicine (n = 4), high-dose prednisone (n = 4), pentoxyphilline (n = 2) and various immunossuppressors and/or immunomodulators given successively: intravenous cyclophosphamide (n = 4), azathioprine (n = 3), interferon alpha (n = 3), cyclosporine A (n = 2), oral cyclophosphamide (n = 1), mycophenolate mofetil (n = 1), methotrexate (n = 1), high-dose immunoglobulin (n = 1). Combination with respectively 1, 3, 4 and 5 immunossuppressors and/or immunomodulators failed before institution of infliximab. After informed consent was obtained, infliximab was administered as a single infusion of 5 mg/kg (maximum dose: 400 mg) at day 1, at week 2, 6 and then every 8 weeks. RESULTS: With a follow-up ranging from 7 to 22 months, infliximab was efficient in all cases. The mean prednisone dose decreased from 45 mg to 13 mg daily. Total recovery of visual acuity was observed in half of the cases. Infliximab was well tolerated without fever, severe sepsis or autoimmune manifestation. CONCLUSION: Infliximab may be efficient in refractory uveitis due to Behcet's disease. The optimal dose, rhythm and duration of infliximab infusions need to be standardized.

Treatment of recalcitrant idiopathic orbital inflammation (chronic orbital myositis) with infliximab. Garrity JA, Coleman AW, Matteson EL, Eggenberger ER, Waitzman DM. Rochester, Minnesota, USA.

Am J Ophthalmol. 2004 Dec; 138 (6): 925 - 30

PURPOSE: To report results of treatment with a monoclonal antibody (infliximab) directed against tumor necrosis factor alpha in seven patients with chronic and difficult-to-control idiopathic orbital inflammation (orbital myositis). DESIGN: Observational case series. METHODS: Retrospective data were collected from seven patients who had idiopathic orbital inflammation and who were evaluated at three medical centers. All patients were treated with infliximab after the failure of traditional therapy, which included corticosteroids, radiotherapy, or anti-inflammatory chemotherapeutic agents. RESULTS: All seven patients had a favorable response to treatment with infliximab. One patient with Behcet disease required supplemental oral corticosteroids. Pain, swelling, and need for concomitant corticosteroids were the primary measures of treatment success. Symptoms of comorbid disease in four patients also improved (Crohn disease in two, Behcet disease in one, and psoriasis in one). There were no untoward effects of treatment after a mean follow-up of 15.7 months (range, 4 to 31 months). CONCLUSIONS: Treatment with infliximab appears to offer another therapeutic option in cases of recalcitrant or recurrent idiopathic orbital inflammation in which conventional treatment fails.

Infliximab for the treatment of posterior uveitis with retinal neovascularization in Behcet disease. Giansanti F, Barbera ML, Virgili G, Pieri B, Emmi L, Menchini U. Firenze, Italy.

Eur J Ophthalmol. 2004 Sep - Oct;14 (5): 445 - 8

PURPOSE: To report a case of posterior uveitis with retinal neovascularization in a patient with Behcet disease treated with infliximab. METHODS: A 50-year-old man with a history of recurrent relapses of ocular inflammation despite immunosuppressive therapy developed retinal neovascularization near the optic disk. The patient was treated with infliximab and followed up for 12 months. RESULTS: Retinal neovascularization regressed 8 months after the first anti-tumor necrosis factor (TNF) treatment and with six infusions of infliximab. The ocular inflammation resolved almost completely. CONCLUSIONS: The result suggests that anti-TNF therapy may be effective in the treatment of retinal neovascularization caused by panuveitis in Behcet disease.

Markomichelakis NN, Theodossiadis PG, Pantelia E, Papaefthimiou S, Theodossiadis GP, Sfikakis PP. Athens, Greece.

Am J Ophthalmol. 2004 Oct; 138 (4): 648 - 50

PURPOSE: To assess the efficacy of the anti-TNF monoclonal antibody infliximab in uveitis patients without clinically evident ocular inflammation and impaired visual acuity because of chronic cystoid macular edema (CME). DESIGN: Prospective, noncomparative, interventional case series. METHODS: Patients with refractory CME (14 eyes, mean duration of 14 months), associated with intermediate uveitis (n = 6), Adamantiades-Behcet disease (n = 2), adult-type vascular pseudotumor (n = 1), and HLAB27+-related uveitis (n = 1) received an intravenous infliximab infusion (5 mg/kg); five patients were retreated after 1 month. RESULTS: Macular thickness, measured by ocular coherence tomography, was reduced from 428 +/- 138 microm to 219 +/- 51 microm at 2 months postbaseline (P = .0001), while visual acuity increased from 0.41 +/- 0.18 to 0.83 +/- 0.17 (P < .00001). Anatomic and functional improvement was sustained at 6 months in all. No ocular or extra-ocular side effects were noted. CONCLUSION: These promising results suggest that TNF may play an important pathogenetic role in chronic CME, thus, a controlled trial is warranted.

Hamuryudan V, Er T, Seyahi E, Akman C, Tuzun H, Fresko I, Yurdakul S, Numan F, Yazici H. Istanbul, Turkey.

American Journal of Medicine 2004 Dec; 117 (11): 867 - 70

Calamia K, Schirmer M, Melikoglu M.

Curr Opin Rheumatol. 2005 Jan;17: 1 - 8

PURPOSE OF REVIEW: Large vessel vasculitis occurs in a subgroup of patients with Behcet disease at high risk for disease-related morbidity and mortality. Recognition of patients at risk, early detection of vasculitis, and the need for aggressive treatment are essential for optimal care of these patients. The authors review the clinical spectrum and management of large vessel problems in Behcet disease, highlighting contributions over the past year. RECENT FINDINGS: Vasculo-Behcet patients are at risk for multiple vessel-related complications including thromboses, stenoses, occlusions, and aneurysms. A number of factors may contribute to thrombosis in individual cases, but the primary reason for clot seems to reside in the inflammatory process in the arterial wall, still incompletely understood. An appreciation for the challenges in the perioperative period requires the joint efforts of physicians and surgeons, and fuels the study of alternate, less invasive procedures for Behcet patients. SUMMARY: Because of earlier recognition, aggressive medical treatment, and novel surgical procedures, the morbidity and mortality of large vessel vasculitis in Behcet disease are beginning to change. In the absence of controlled treatment studies, reports of clinical experience remain an important source of information for clinicians. Identification of patients at risk for vascular complications remains a priority.

Allali F, Benomar A, Karim A, Lazrak N, Mohcine Z, El Yahyaoui M, Chkili T, Hajjaj-Hassouni N. Sale, Morocco.

Scand J Rheumatol. 2004; 33 (5): 362 - 3

We report 12 cases of Behcet's disease (BD) in children. The mean age of symptom onset was 12.4 years. Four patients (33.3%) had a past familial history of BD. Clinical manifestations were: oral aphtosis (n = 12), genital aphtosis (n = 9), ocular involvement (n = 9), neuro-Behcet (n = 6), venous thrombosis (n = 4), articular involvement (n = 3), and entero-Behcet (n = 1). All patients but one were initially treated with steroids; three cases with ocular involvement were treated with chlorambucil; and three other cases of neuro-Behcet were treated with cyclophosphamide. After a mean follow-up of 4 years, four patients with neurological involvement developed steroid-dependence with recurrence of symptoms. Four patients had optic atrophy with blindness.

Aouba A, Nebie L, Fabiani JN, Bruneval P, Patri B, De Bandt M. Paris, France

Presse Medicale. 2004 nov; 33 (19 Pt 2):1367 - 9

INTRODUCTION: Aseptic endocarditis or/and endomyocardial fibrosis are rarely reported in Behcet's disease. OBSERVATION: We report on a case of a 21-year-old man living in Algeria, revealed by verrucous tricuspid valvulitis extending to the ventricular endomyocardium and complicated with right heart failure, initially misdiagnosed and treated as infective endocarditis occurring on rheumatic cardiac after-effects. DISCUSSION; We discuss the lack of specificity of Jones criteria and emphasize the need to include cardiac involvement in Behcet's disease in the differential diagnosis of rheumatic fever carditis. This message is notably important in some countries where the prevalence of these two entities are among the highest in the world.