Listed during April - October 2005

Each 6 months, we present a list of all clinical reports with abstracts, when available, that have been published on Behcet’s disease, found in our search of the Medline database. On each occasion we invite a reviewer to focus on one group of these papers and comment on the material, as was done so well six months ago byM. Zierhut. This time we have chosen to prepare this comment ourselves, highlighting current reports on anti-TNFa therapies, reviewing part of the short history of use of these agents in Behcet’s disease (BD), with some editorial comment. We welcome any comments which we may be able to publish next time. We are also interested if any readers would like to participate in this activity with future reviews. This effort is work-in-progress and can evolve into any scheme which can provide value to readers. Do take advantage of the opportunity to review the listing of articles published, those in the clinical as well as the non-clinical list. Almost certainly you will find articles of interest among the wealth of publications.

Like rheumatoid arthritis and Crohn’s disease, BD is believed to be associated with a Th1-mediated immune response. Increased levels of TNFa are found in BD which provides support for the empirical use of anti-TNFa therapies used in a number of published cases and small case series in recent years.

The earliest reports of the efficacy of anti-TNFa therapies in BD examined the effects on gastrointestinal and mucocutaneous manifestations. Rapid and dramatic improvement in gastrointestinal and other symptoms of the disorder after treatment with infliximab was reported. Resistant mucocutaneous symptoms improved dramatically. Even central nervous system disease has been targeted successfully by anti-TNFa treatment. Overall a large number of case reports and studies on anti-TNFa therapies in BD, both in adults and children, have been reported or presented at recent Annual European Congresses of Rheumatology (EULAR), at the International Conferences on Behçet’s Disease (ISBD), and at meetings of the American College of Rheumatology (ACR).

In particular, experience with anti-TNFa treatments for the ocular manifestations of BD has been growing and the results are very encouraging. Sfikakis (Sfikakis PP et al. Lancet 358(9278):295-6, 2001) reported the benefits of infliximab in 5 patients with panuveitis in BD. A review in 2002, at which time more than 80 patients from 10 different countries had received anti-TNFa treatment, examined the evidence to support a role for anti-TNFa therapies in the disorder (Sfikakis PP, Ann Rheum Dis 61 Suppl 2:ii51-3, 2002). In 2004 the positive experience of Greek investigators using a single infusion of infliximab at the time of an ocular flare was reported (Sfikakis PP et al. Ann Intern Med 140(5):404-6, 2004). The long awaited positive Japanese experience with infliximab in the treatment of cyclosporine resistant ocular BD has now been reported in an open study of 13 patients (Ohno S. et al. J Rheumatol 31(7):1362-8, 2004; Nakamura S et al. Intern Ophthalmol Clin 45(2):179-89, 2005), as has the experience of the French in a group of 4 patients (Wechsler B et al. Clin Exp Rheumatol 22(4 Suppl 34):S14-6, 2004).

In the last 6 months, more reports on the efficacy of anti-TNFa therapies in BD have been published. These include open reports by Lindstedt and by Baughman of the use of infliximab in ocular inflammatory disorders including BD, the former calling for a controlled trial. A placebo controlled trial using etanercept in BD has been reported by Melikoglu, Fresko et al. from Istanbul, and is listed among the current publications (Melikoglu M. et al. J Rheumatol 32(1):98-105, 2005). This clearly demonstrates the efficacy of etanercept for mucocutaneous BD and is the only controlled trial reported to date.

Has the opportunity for a larger controlled trial of anti-TNFa agents in the treatment of ocular BD been lost? Is the uncontrolled data so compelling that a controlled trial is unnecessary, or even unethical? Despite the strong but uncontrolled data for ?-interferon, it was decided to carry out a controlled trial comparing this agent to the standard of care. If this trial supports the efficacy of interferon, how will we compare this treatment to anti-TNFa agents? Do both the monoclonal antibodies and etanercept work for BD? There are many questions yet to be answered. It is not clear that pharmaceutical companies would provide the support to carry out these studies for a disease that is limited in distribution, largely to areas where their return on investment cannot be realized.

The use of anti-TNFa therapies in rheumatic diseases has been of tremendous benefit for many patients and a source of enthusiasm and excitement for rheumatologists who treat these patients. While the short term, and potential long term side effects, of these agents continue to be a concern and require continued vigilance, the risk-benefit ratio of these agents in the treatment of serious diseases appears acceptable. However, our wish that these agents would be useful in all disorders treated with corticosteroids and in diseases requiring more toxic immunosuppressive drugs is fading with the publication of some recent data. Despite multiple anecdotal reports, the negative result of the Wegener’s Granulomatosis Etanercept Trial and the results of a trial of infliximab in giant cell arteritis (Hoffman, 2005) have demonstrated that controlled trials are the gold standard for proof of efficacy of therapeutic agents.

There should be little hesitation to consider infliximab for patients with uncontrolled disease who have failed conventional therapy. We believe that controlled studies should be done in earlier ocular BD, so that the proper place of this treatment can be better understood.

Atzeni F, Sarzi-Puttini P, Doria A, Iaccarino L, Capsoni F. Autoimmunity Reviews. 4(3):144-52, 2005.

TNF-alpha is a crucial cytokine in the establishment and maintenance of inflammation in multiple autoimmune and non-autoimmune disorders. A number of large placebo-controlled trials have shown that infliximab, a chimeric monoclonal antibody against TNF-alpha, is effective and well-tolerated in patients with Crohn's disease and rheumatoid arthritis (RA) and has become a widely used treatment for these diseases. More recent controlled trials have also shown the effectiveness of TNF-alpha blockers in psoriasis, psoriatic arthritis, and ankylosing spondylitis. The results of clinical trials, open-label studies, and case studies indicate that TNF inhibitors (alone or in combination with other protocols) look very promising for the treatment of a variety of other conditions, including uveitis, sarcoidosis, Sjogren's syndrome (SS), Behcet's syndrome, vasculitis, and graft versus host disease. There is a rationale for using TNF blockade even in systemic lupus erythematosus, a prototype of autoantibody-mediated disease, and a pilot study seems to confirm this potential effective approach. The neutralisation of TNF might therefore play a role in the treatment of many autoimmune and non-autoimmune disorders other than Crohn's disease or RA. We here review the current and prospective roles of infliximab in the treatment of autoimmune diseases and other conditions that do not currently have FDA or EMEA approval. [References: 40]

Management of sight-threatening uveitis: new therapeutic options. [Review]

Becker MD, Smith JR, Max R, Fiehn C. Drugs. 65(4):497-519, 2005.

Over the past 2 decades therapy for the treatment of intraocular inflammation (uveitis) has developed into a highly differentiated approach with an increasing number of drug options. This paper primarily summarises literature from the past 5 years (2000 to May 2004), gives an update on systemic immunosuppressive therapy for non-infectious uveitis and speculates about new developments that could become relevant in the near future for the treatment of uveitis patients. The spectrum of immunosuppressive drugs has been notably expanded by tumor necrosis factor inhibitors, but with some limitations to uveitis. Behcet's disease is an example of uveitis where a multisystem disorder can affect the eye very severely. This clinical example has been used to investigate the utility of many different types of immunosuppressive therapies and the clinical approach is extensively discussed in this review. An accompanying table summarises the proposed mode of action, standard dosage, common adverse effects, as well as estimated cost of current treatment options. [References: 137]

Anti-TNF-alpha therapy for sight threatening uveitis.

Lindstedt EW, Baarsma GS, Kuijpers RW, van Hagen PM.British Journal of Ophthalmology. 89(5):533-6, 2005.

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.

Conti F, Priori R, Chimenti MS, Coari G, Annovazzi A, Valesini G, Signore A. Arthritis & Rheumatism.52(4):1224-6, 2005.

Positive experiences with intraarticular infliximab have been reported in patients with rheumatoid arthritis, ankylosing spondylitis, and Behcet's disease. We used intraarticular infliximab to treat resistant knee monarthritis in a patient with spondylarthropathy. Clinical and laboratory improvement was associated with improvement in scintigraphic findings. This approach is less expensive than intravenous administration of infliximab. We suggest that selection of candidates for this innovative therapy should be guided by anti-tumor necrosis factor alpha scintigraphy.

Successful treatment of long-standing neuro-Behcet's disease with infliximab.

Sarwar H, McGrath H Jr, Espinoza LR. Journal of Rheumatology. 32(1):181-3, 2005.

Behcet's disease is a chronic relapsing form of vasculitis affecting multiple organs. Central nervous system involvement is a common presentation. Tumor necrosis factor-alpha (TNF-alpha) is considered to play a major role in the disease pathogenesis. We describe a patient with a long history of neuro-Behcet's disease who showed a remarkable response to infliximab therapy. Given the variable and limited success of other interventions, the use of anti-TNF-alpha therapy seems to be effective for patients with refractory disease.

Melikoglu M, Fresko I, Mat C, Ozyazgan Y, Gogus F, Yurdakul S, Hamuryudan V, Yazici H. Journal of Rheumatology. 32(1):98-105, 2005.

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.

Baughman RP, Bradley DA, Lower EE. International Journal of Clinical Pharmacology & Therapeutics. 43(1):7-11, 2005.

OBJECTIVE: Infliximab is a chimeric antibody which binds tumor necrosis factor (TNF). It is effective in several chronic inflammatory conditions, including sarcoidosis. METHODS: We report our experience with infliximab in chronic ocular inflammation as part of a retrospective review of all patients treated for chronic inflammatory ocular conditions seen over a 2-year period at our institution. RESULTS: 14 patients with various underlying ocular conditions were treated during the previous two years including patients with sarcoidosis (7), Crohn's disease (2), birdshot choroiditis (2), idiopathic disease (2), Volt-Koyanagi-Harada (1) and Behcet's disease (1). All patients had persistent inflammation despite systemic immunosuppressive agents and all but one patient experienced marked improvement in ocular inflammation with infliximab. One patient was non-compliant and non-evaluable; four patients, who had previously received etanercept with either no response (3 patients) or subsequent relapse (1 patient), responded to infliximab. CONCLUSION: Infliximab is an effective therapy in chronic inflammatory eye disease, especially when related to sarcoidosis.

Arayssi T, Hamra R, Homeidan F, Uthman I, Awwad ST, Mroue K, Mansour W, Bashshur ZF. Clinical & Experimental Rheumatology. 23(3):427, 2005.

Anti-tumor necrosis factor alpha antibody in the treatment of Behcet's disease.

Nakamura S, Ohno S

International Ophthalmology Clinics. 45(2):179-89, 2005.

Yamauchi Y, Cruz JM, Kaplan HJ, Goto H, Sakai J, Usui M. Ocular Immunology & Inflammation.13(4):317-25, 2005.

AIM: To report a 47-year-old Japanese woman with a one-year history of Behcet's disease who complained of sudden bilateral visual loss with concurrent anterior ischemic optic neuropathy (AION). CASE REPORT: The patient's Snellen visual acuity was 0.1 (OD) and 0.3 (OS) of onset. There was bilateral mild anterior chamber inflammation. Bilateral optic disc pale swelling was observed without retinal exudates and edema. Fluorescein angiography demonstrated bilateral hypofluorescence of the optic disc in early frames but with no distinct retinal vasculitis. Visual field showed bilateral relative central scotoma and right altitudinal hemianopsia. Laboratory examination revealed an ESR of 26 mm in the first hour with a C-reactive protein level of < 0.3 mg/dl. Periocular injection of triamcinolone acetonide in both eyes without systemic corticosteroid administration improved her visual acuity to 0.7 (OD) and 1.2 (OS) within 45 days of onset. Bilateral optic disc swelling gradually resolved. In the early stages, fluorescein angiography demonstrated normal optic disc filling in both eyes. There was a residual right central scotoma on visual field. CONCLUSION: We observed an extremely rare case of simultaneous bilateral AION with Behcet's disease with marked visual recovery within 45 days of onset.

Intravitreal triamcinolone acetonide for treatment of intraocular proliferative, exudative, and neovascular diseases. [Review]

Jonas JB, Kreissig I, Degenring R. Progress in Retinal & Eye Research. 24(5):587-611, 2005.

Within the last three years, triamcinolone acetonide has increasingly been applied intravitreally as treatment option for various intraocular neovascular edematous and proliferative disorders. The best response in terms of gain in visual acuity after the intravitreal injection of triamcinolone acetonide was found in eyes with intraretinal edematous diseases such as diffuse diabetic macular edema, branch retinal vein occlusion, central retinal vein occlusion, and pseudophakic cystoid macular edema. Visual acuity increased and degree of intraocular inflammation decreased in eyes with various types of non-infectious uveitis including acute or chronic sympathetic ophthalmia and Adamantiadis-Behcet's disease. Intravitreal triamcinolone may be useful as angiostatic therapy in eyes with iris neovascularization and proliferative ischemic retinopathies. Possibly, intravitreal triamcinolone may be helpful as adjunct therapy for exudative age-related macular degeneration, possibly in combination with photodynamic therapy. In eyes with chronic, therapy resistant, ocular hypotony, intravitreal triamcinolone can induce an increase in intraocular pressure and may stabilize the eye. The complications of intravitreal triamcinolone therapy include secondary ocular hypertension in about 40% of the eyes injected, cataractogenesis, postoperative infectious and non-infectious endophthalmitis, and pseudo-endophthalmitis. Intravitreal triamcinolone injection can be combined with other intraocular surgeries including cataract surgery. Cataract surgery performed some months after the injection does not show a markedly elevated rate of complications. If vision increases and eventually decreases again after an intravitreal triamcinolone acetonide injection, the injection can be repeated. The duration of the effect of a single intravitreal injection of triamcinolone depended on the dosage given. Given in a dosage of about 20mg to non-vitrectomized eyes, the duration of the effect and of the side-effects was 6-9 months. Intravitrea triamcinolone acetonide may offer a possibility for adjunctive treatment of intraocular edematous and neovascular disorders. One has to take into account the side-effects and the lack of long-term follow-up observations. [References: 214]

Pars plana vitrectomy for ocular complications of Behcet's disease.

Sullu Y, Alotaiby H, Beden U, Erkan D. Ophthalmic Surgery, Lasers & Imaging. 36(4):292-7, 2005.

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.

Guler I, Ubeyli ED. Computers in Biology & Medicine. 35(2):121-32, 2005.

Doppler ultrasound is known as a reliable technique, which demonstrates the flow characteristics and resistance of ophthalmic arteries. In this study, ophthalmic arterial Doppler signals were obtained from 106 subjects, 54 of whom suffered from ocular Behcet disease while the rest were healthy subjects. Multilayer perceptron neural network (MLPNN) employing delta-bar-delta training algorithm was used to detect the presence of ocular Behcet disease. Spectral analysis of the ophthalmic arterial Doppler signals was performed by least squares (LS) autoregressive (AR) method for determining the MLPNN inputs. The MLPNN was trained with training set, cross validated with cross validation set and tested with testing set. All these data sets were obtained from ophthalmic arteries of healthy subjects and subjects suffering from ocular Behcet disease. Performance indicators and statistical measures were used for evaluating the MLPNN. The correct classification rate was 96.43% for healthy subjects and 93.75% for unhealthy subjects suffering from ocular Behcet disease. The classification results showed that the MLPNN employing delta-bar-delta training algorithm was effective to detect the ophthalmic arterial Doppler signals with Behcet disease.

Matsuo T, Itami M. Ocular Immunology & Inflammation. 13(1):73-7, 2005.

PURPOSE: The aim of this study was to note the clinical features of patients with Behcet's disease who did not show recurrent episodes of eye involvement during long-term follow-up. METHODS: A retrospective review was made of 70 Japanese patients with Behcet's disease who were seen during a 23-year period from 1974 to 1996 and followed for five or more years at Okayama University Hospital. The 70 patients were divided into three groups: 36 patients with recurrent episodes of eye involvement; 13 patients with non-recurrent one-time only eye involvement; and 21 patients with no eye involvement at all during a follow-up period of five or more years. Clinical features were compared between these three groups. RESULTS: Female sex (p = 0.0263, chi-square test), young age at the onset (p = 0.0322, Kruskal-Wallis test), and genital ulceration (p = 0.0003, chi-square test) were significantly associated with the non-recurrent or no eye involvement patients. CONCLUSIONS: Female patie!

nts with onset at a young age and who show the three signs of skin lesions and oral and genital ulcerations may have non-recurrent one-time only or no eye involvement at follow-up. A better visual prognosis can be expected for these patients.

Gedik S, Akova Y, Yilmaz G, Bozbeyoglu S. Ocular Immunology & Inflammation. 13(1):51-8, 2005.

PURPOSE: To define the indocyanine green (ICG) and fundus fluorescein angiographic (FFA) features of Behcet's disease. METHODS: The study included 49 eyes of 25 patients (19 males, 6 females; mean age: 34.1 years; age range: 14-68 years) with Behcet's disease and active ocular involvement that fulfilled the criteria of the International Study Group for Behcet's Disease. In each patient, FFA and ICG angiography were performed consecutively in the same session using a TRC-50I/A fundus camera (Topcon, Tokyo, Japan) coupled to an Image Net digitizing system. All images were analyzed and FFA, ICG, and clinical findings summarized. RESULTS: The mean duration of ocular involvement was 52.4 months (range: 2-240 months). FFA showed staining and dye leakage at the optic disc in 44 (89.8%) eyes and diffuse vasculitis in 36 (73.5%) eyes. Macular edema and ischemia were observed in 31 (63.3%) and 3 (6.1%) eyes, respectively. Eight (16.3%) eyes exhibited detectable retinal FFA alteratioins, but no abnormalities on ICG angiography. Disc ICG hyperfluorescence was observed in 23 (46.9%) eyes. ICG angiography revealed choroidal fuzziness in 16 (32.6%) eyes, hyperfluorescent spots in 13 (26.5%) eyes, and hypofluorescent plaques in 12 (24.5%) eyes. Eleven eyes (22.4%) showed no abnormal findings on ICG angiography. CONCLUSION: ICG and FFA complement each other as tools for diagnosing patients with Behcet's disease. FFA often serves as a fairly reliable guide. Although some ICG findings cannot be demonstrated by FFA, they are not specific or pathognomonic. We do not recommend performing both procedures for Behcet's disease.

Causes of uveitis in a referral hospital in Ankara, Turkey.

Sengun A, Karadag R, Karakurt A, Saricaoglu MS, Abdik O, Hasiripi H. Ocular Immunology & Inflammation. 13(1):45-50, 2005.

PURPOSE: To evaluate the etiology of uveitis and its associated systemic findings in a referral hospital in Ankara, Turkey. MATERIALS AND METHODS: Clinical records of patients in our uveitis department were reviewed retrospectively and 419 eyes of 300 consecutive patients were included in the study. Cases of uveitis due to postoperative endophthalmitis, perforating injuries, and foreign bodies were excluded from the study. The data, including sex, age, clinical presentation, disease course and outcome, ocular involvement, etiology, and associated systemic findings, were entered into a computer database. Pearson chi-square statistical analysis was performed to analyze the data whenever necessary. RESULTS: The study subjects consisted of 162 males and 138 females, with a female to male ratio of 1:1.17 and an average age of 35.7 years. Anterior uveitis was the most common form of uveitis with 43.6%, followed by posterior uveitis (26.6%), panuveitis (20.6%), and intermediate uveitis (9%). Idiopathic uveitis (28.3%) was the most frequent cause of uveitis diagnosed, followed by Behcet's disease (26%) and rheumatic diseases (9%). In 215 (71.7%) out of 300 patients, a specific cause of uveitis with associated systemic findings was identified. Uveitis was attributed to an infectious etiology in only 41 patients (13.6%) of the entire study population. COMMENTS: The etiology of uveitis and its associated systemic findings may vary depending on the geographic distribution of patients and local factors. These results represent those of patients with uveitis referred to hospitals in central Anatolia.

Sheu SJ, Yang CA. Kaohsiung Journal of Medical Sciences. 20(11):558-62, 2004.

Behcet's disease is an inflammatory disorder of unknown cause, characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. Ocular involvement occurs in 60-80% of patients with Behcet's disease and presents as panuveitis in most cases. Posterior segment involvement may lead to irreversible alterations and significant vision loss. The development of a partial or full-thickness macular hole, though rarely reported, may cause serious vision loss. In this report, we present two cases of macular hole in the worse eye of bilateral cases of Behcet's disease, and discuss the possible mechanisms and management in such cases.

Tarzi MD, Lightman S, Longhurst HJ. Rheumatology. 44(7):953-4, 2005.

Case Reports. (Letter)

Sakellariou G, Berberidis C, Vounotrypidis P.

Rheumatology. 44(2):258-60, 2005.

Serous detachment of macula in Behcet disease.

Ozdemir H, Mudun B, Karacorlu M, Karacorlu S. Retina. 25(3):361-2, 2005.

Case Report. Journal Article.

Behcet's disease, associated with subarachnoidal hemorrhage due to intracranial aneurysm.

Zsigmond P, Bobinski L, Bostrom S. Acta Neurochirurgica. 147(5):569-71; discussion 571, 2005.

Behcet's disease is an unusual medical condition in central Europe and North America, however more common in Turkey and Japan. It was originally described in Turkey, characterized by recurrent oral ulcers, genital ulcers and also uveitis. A variety of vascular lesions such as venous occlusions, arterial aneurysms and varices account for the high rate of morbidity and mortality with this disease. Arterial aneurysms most commonly occur in the abdominal aorta, femoral arteries and in the pulmonary arteries.To our knowledge there have been seventeen documented reports of patient's with Behcet's disease combined with aneurysms of cerebral arteries. We describe a patient with Behcet's disease and subarachnoid haemorrhage due to a ruptured cerebral aneurysm.

Ishida C, Furui E, Sakashita Y, Yamada M. Internal Medicine. 44(4):326-7, 2005.

We report a case of a 53-year-old man with Behcet's disease (BD) and paradoxical stroke. This patient suddenly developed sensory aphasia, right oculomotor palsy, nystagmus, and left ataxic hemiparesis. MR images showed fresh embolic infarcts in the midbrain and the left parietotemporal lobe. He presented with uveitis, oral aphthous ulcers and genital ulcers. An electrocardiogram revealed no abnormalities, and a transesophageal echocardiogram disclosed a patent foramen ovale. Since venous involvement is common in BD, we suspected the presence of smaller thrombi probable in this patient. We conclude that embolic stroke is a notable complication of BD with right-to-left shunting.

Magnetic resonance spectroscopic findings in neuro-Behcet disease.

Scully SE, Stebner FC, Yoest SM. Neurologist. 10(6):323-6, 2004.

BACKGROUND: Behcet disease is a heterogeneous, multisystem disorder with elements of both environmental and autoimmune pathologic processes. Neuro-Behcet, found in 10%-25% of Behcet patients, has been characterized as a vasculitis primarily affecting the brain stem. On magnetic resonance imaging, some cases demonstrate extension to the bilateral cerebral cortices and/or the spinal cord, often with a degree of mass effect. Content of this paper provides a case study, along with review of the literature regarding this disease entity, and proceeds to outline the appearance of neuro-Behcet on magnetic resonance spectroscopy. CONCLUSION: In essence, a drop in the N-acetyl aspartate peak indicative of axonal loss in concert with elevations in lipid myoinositol and choline/creatine ratios (nonspecific markers of myelin breakdown) is found, indicating that a possible demyelinating process is complexed with the vasculitic pathology neuro-Behcet is traditionally ascribed to.

Tohoku Journal of Experimental Medicine. 206(1):15-22, 2005.

Behcet's disease (BD) is a multisystem inflammatory disorder characterized by recurrent oral and genital ulcers and uveitis. BD patients without neurological involvement frequently have mild neurological symptoms. The aim of this study was to evaluate whether BD patients without neurological involvement have any changes in cognitive functions. Twenty BD patients without neurological involvement and 13 control subjects were included in the study and were analyzed by neurophysiological and neuropsychological examinations. The cognitive event-related potentials (P300) were recorded from the frontal, central and parietal areas of the right and left hemispheres of the patients and control subjects. Likewise, all individuals were evaluated with neuropsychological tests. In contrast to a study with similar design, we did not find any difference between the cognitive event-related potentials values of BD patients without neurological involvement and the control subjects. All BD patients without neurological involvement exhibit normal results of the neuropsychological test. In conclusion, the results of neuropsychological tests and cognitive event-related potentials values in BD patients without neurological involvement are indistinguishable from those in control subjects and no alteration of cognitive functions is present.

Manifestations of Neuro-Behcet's disease. Report of two cases and review of the literature. [Review]

Sour Ho CL, Deruytter MJ. Clinical Neurology & Neurosurgery. 107(4):310-4, 2005.

Behcet's disease (BD) is a rare condition, more commonly seen in Turkey, the Middle East, the Mediterranean, and the Far Eastern countries. It is a multifaced disease involving the central nervous system (CNS), skin, joints, intestines and blood vessels. CNS involvement occurs in 10-25% of the patients with BD. We report two cases with Neuro-Behcet's disease. Both patients were females and they were in their second decade of life (early forties) when they suffered from relapsing retinitis and uveitis which led to blindness, despite treatment with steroids. They presented with clinical signs of brainstem syndrome, meningoencephalitis and the initial radiological investigations of our patients revealed encephalitic lesions in the brainstem. In the first case, an intracerebral abscess developed 4 years later, and it was evacuated under stereotactic guidance. The most common neuropathologic findings in Neuro-Behcet's disease are focal necrotic lesions in the brain. However, the association of Behcet's disease (BD) with cerebral abscess is very rare. There were only three reported cases of such. Ten years after the initial diagnosis of BD, our second patient suffered from subarachnoid hemorrhage due to a ruptured aneurysm of the superior cerebellar artery, which was then resected successfully. Intracranial aneurysm in patients with Behcet's disease has previously been documented in eight patients. To our knowledge, this is the only case of Neuro-Behcet's disease associated with an aneurysm of the superior cerebellar artery. [References: 22]

Anik Y, Kural Z, Demirci A, Akansel G, Aksu S, Vural M. Neuroradiology. 47(2):108-13, 2005.

The aim of this study was to determine the contribution of magnetization transfer ratios (MTRs) in detecting disease in normal-appearing brain regions of patients with neuro-Behcet (NB) disease. Thirty-two patients with NB disease were assessed. Fifteen healthy volunteers were examined as the control group. Magnetic resonance (MR) imaging of the head was performed without and with magnetization transfer (MT) contrast. Signal intensity measurements were obtained from ten anatomical regions (centrum semiovale, corona radiata, internal capsule, forceps major, forceps minor, thalamus, substantia nigra pars compacta, substantia nigra pars grisea, inferior pons and middle cerebellar peduncle) in both groups. Also measured in the NB group were parenchymal lesions in the brain stem, basal ganglia and cerebral deep white matter. MTR was calculated for each measurement. Statistical analysis was performed with Mann-Whitney U and independent t-tests with computer-based SPSS 11.0 for W!

indows software. A P value below 0.05 was considered statistically significant. The mean MTR of the parenchymal lesions in the NB group was lower than the mean MTR of the normal-appearing parenchyma in both the NB patients and the normal group. For the normal-appearing parenchyma the mean MTR in the NB group was higher than that for the controls for all regions except the corona radiata; however, the difference was statistically significant only for the thalamus. The MRI-visible parenchymal involvement of Behcet's disease causes a decrease in MTR. For the normal-appearing brain, although lacking statistical significance for the most regions studied, the tendency for higher MTR in NB patients compared with controls may offer an insight into the pathophysiology of Behcet's disease.

Spontaneous pseudoaneurysm of the vertebral artery in Behcet's disease. [Review]

Gurer O, Yapici F, Enc Y, Cinar B, Ozler A. Annals of Vascular Surgery. 19(2):280-3, 2005.

A pulsatile mass and severe neck pain developed suddenly in a 15-year-old female patient suffering from Behcet's disease. Magnetic resonance imaging showed a pseudoaneurysm at the C3-C4 level that was 51 x 49 x 45 mm in size, originating from the left vertebral artery, with a thin neck and thrombus inside. Repair of the vertebral artery wall by percutaneous transluminal intervention was not successful. Because of the possibility of rupture, the patient underwent surgical repair. Ligation of the left vertebral artery was applied 1 cm above the origin of the subclavian artery. During the subsequent postoperative period, no further complications were seen. From our review of the literature, this is the first reported case of surgical treatment of spontaneous development of a pseudoaneurysm at the vertebral artery in association with Behcet's disease. Ligation of the vertebral artery can be safely used to control a pseudoaneurysm related to Behcet's disease. [References: 10]

Transverse myelitis in a patient with Behcet's disease: favorable outcome with a combination of interferon-alpha.

Calguneri M, Onat AM, Ozturk MA, Ozcakar L, Ureten K, Akdogan A, Ertenli I. Kiraz S Clinical Rheumatology. 24(1):64-6, 2005.

We report here on a 24-year-old patient with Behcet's disease who had been diagnosed with acute transverse myelitis. He was successfully treated with a combination regimen of a steroids, cyclophosphamide, and interferon-alpha. The treatment strategy with specific emphasis on interferon-alpha is discussed in the light of the pertinent literature.

Cadoni G, Agostino S, Manganelli C, Scipione S, Turco S, Focosi F, Ottaviani F. Acta Otorhinolaryngologica Italica. 24(5):262-6, 2004.

Few reports have appeared in the literature concerning vestibular findings in Behcet's disease. In the present study, extensive vestibular testing, performed in 14 patients (8 male, 6 female; mean age: 32 years; range: 12-51) presenting definite Behcet's disease, revealed a high prevalence of central vestibular dysfunctions (78%). Data reported here suggest that an otoneurological evaluation of Behcet's disease patients may be helpful in identifying unexpected vestibular dysfunctions and central nervous system involvement different from the classical manifestations of the neuro-Behcet's syndrome.

Mitsui Y, Mitsui M, Urakami R, Kihara M, Takahashi M, Kusunoki S.Internal Medicine. 44(2):149-52, 2005.

A 53-year-old man with Behcet disease was treated with conventional cyclosporin A (CyA), because of refractory bilateral uveitis. Immediately following the conversion from conventional CyA to a microemulsion formulation, he presented with neurological complications. The neurological findings, pleocytosis of the cerebrospinal fluid (CSF) and brainstem lesions revealed by brain magnetic resonance imaging (MRI) suggested neuro-Behcet disease. After discontinuing CyA and introducing oral prednisolone, the neurological symptoms, pleocytosis of CSF and brainstem lesions on MRI improved. Although the microemulsion formulation, which can maintain a stable level of blood CyA, is a useful agent for the control of ocular lesions in Behcet disease, the resulting abrupt increase in blood CyA level may have induced neuro-Behcet disease.

Matsuo K, Yamada K, Nakajima K, Nakagawa M. American Journal of Neuroradiology. 26(3):650-3, 2005.

Neuro-Behcet disease is one of the clinical forms of Behcet disease. We report a case of neuro-Behcet disease mimicked a brain tumor. This case was initially considered as a brain tumor from mass lesion with edema at left basal ganglia on radiologic images. The lesion, however, was not neoplasia by pathologic diagnosis. By using steroid therapy, the size had been markedly shrunken on the follow-up images. The clinical symptoms were also improved.

Neuro-Behcet disease. A review. [Review]

Borhani Haghighi A, Pourmand R, Nikseresht AR.Neurologist. 11(2):80-9, 2005.

BACKGROUND: Behcet disease is a vasculitis with mucocutaneous, ocular, arthritic, vascular, and other manifestations. Its neurologic manifestations (neuro-Behcet disease) are relatively rare, but they must be thoroughly investigated due to their grave prognosis. REVIEW SUMMARY: The frequency of neurologic manifestations, more common in male Behcet patients, is between 5% and 30%. Both the central and peripheral nervous systems can be involved. Central nervous system manifestations can be divided into 2 main groups: (1) parenchymal involvement, which includes brainstem involvement, hemispheric manifestations, spinal cord lesions, and meningoencephalitic presentations; (2) nonparenchymal involvement, including dural sinus thrombosis, arterial occlusion, and/or aneurysms. Peripheral neuropathy and myopathy are relatively rare. Cerebrospinal fluid analysis reveals pleocytosis and elevated protein levels. Magnetic resonance imaging is the investigation of choice which often reveals iso-/hypointense lesions in T1-weighted images and hyperintense lesions in T2-weighted images, mostly in the mesodiencephalic junction, cerebellar peduncles, and other parts of the brainstem. Corticosteroids and adjuvant immunosuppressive therapy are used for parenchymal manifestations, and corticosteroids and anticoagulants are used for treatment of dural sinus thrombosis. CONCLUSION: Neuro-Behcet disease must be considered in the differential diagnosis of stroke in young adults, multiple sclerosis, movement disorders, intracranial hypertension, intracranial sinovenous occlusive diseases, and other neurologic syndromes.

Turkolmez S, Gokcora N, Alkan M, Gorer MA. Annals of Nuclear Medicine. 19(3):201-6, 2005.

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.

Hosaka A, Miyata T, Shigematsu H, Shigematsu K, Okamoto H, Ishii S, Miyahara T, Yamamoto K, Akagi D, Nagayoshi M, Nagawa H. Journal of Vascular Surgery. 42(1):116-21, 2005.

OBJECTIVE: Surgical treatment of arterial lesions associated withBehcet 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.

Uzun L, Ugur MB, Ulukent SC, Ozdemir H, Koca R. Tohoku Journal of Experimental Medicine. 206(1):81-4, 2005.

This article presents a case of Behcet's Disease (BD) with vascular involvement of the neck, which mimicks a metastatic neck mass in the initial presentation. A 58-year-old man presented with dysphagia, weight loss, bulging on the lateral wall of the left pyriform sinus, and a firm and fixated neck mass suggestive of metastasis. Computed tomography of the neck demonstrated a solid mass, around the bifurcation of the carotid artery together with a pseudoaneurysm of the left external carotid artery. The mass was about four centimeters in diameter and extended to hypopharynx medially. Biopsy from neck mass and hypopharynx revealed no specific pathology. During follow-up the firm and fixated mass changed into a completely pulsatile one in the following three weeks. Reassessment of the patient's past history in detail revealed that he had had recurrent oro-genital ulcers, arthralgia and recurrent skin lesions. The pathergy test was positive. The patient was diagnosed to be BD and treatment consisting of colchicine 1 mg/day, peroral was started. He had a favorable outcome after treatment and was asymptomatic at follow-up of 24 months. It is unusual for BD to present as a neck mass but yet it must be considered in the differential diagnosis of neck masses. The present case report demonstrates how such a mass may mimic metastatic tumoral involvement and cause diagnostic dilemma.

Uzun O. Chest. 127(6):2243-53, 2005.

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 ra! 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. [References: 148]

Sismanoglu M, Omeroglu SN, Mansuroglu D, Ardal H, Erentug V, Kaya E, Guler M, Ipek G, Yakut C.Journal of Cardiac Surgery. 20(2):160-3, 2005.

There is a high frequency of pseudoaneurysm formation in patients with Behcet's disease and their inflammed and fragile tissues are difficult to manipulate. Five patients with Behcet's disease were referred to our cardiovascular surgery department for coronary artery bypass grafting (CABG). Three of them were operated and two were treated medically. Patients that were managed medically had left anterior descending (LAD) lesions below 80% and their stable angina pectoris responded well to medication. There was no early mortality and morbidity. One patient developed pseudoaneurysm of ascending aorta and femoral artery. This patient died in the late postoperative period. At follow-up the operated patients were in Canadian Cardiovascular Society (CCS) Class I, while the medically treated patients were in CCS Class II. Mean follow-up period was 41 +/- 36.21 months. Coronary artery disease (CAD) is extremely rare detected in patients with Behcet's disease. The affected patients are usually young males. Coronary artery bypass grafting is also rarely performed in these patients and long-term results of such operations are not available in the literature. We present five patients with Behcet's disease that had CAD, three operated and two medically treated, and report their long-term results. We suggest a conservative approach in patients with Behcet's disease because of the high risk of pseudoaneurysm formation in the postoperative period. If CABG cannot be avoided we recommend operating the patients on the beating heart with minimal aortic manipulation.

Ates A, Aydintug O, Olmez U, Duzgun N, Duman M. Rheumatology International. 25(1):42-4, 2005.

OBJECTIVE: Behcet's disease (BD) is a multisystemic inflammatory disorder of unknown etiology that is sometimes associated with thrombosis. However, the mechanism of hypercoagulability is not known. In this study, we investigated whether hyperhomocysteinemia, being a well-known risk factor for thrombosis, is also a contributing risk factor to venous and arterial thromboses of BD. METHODS: Forty-five patients with BD and 40 healthy subjects were included in the study. Sixteen patients had vascular involvement. Serum homocysteine levels were determined by fluorescence polarization immunoassay. RESULTS: In male patients, the frequency of vascular involvement was significantly higher than in females (46.7% vs 13.3%, P < 0.05). Serum homocysteine levels were significantly higher in patients with BD than healthy controls (P < 0.01), in patients with vascular involvement than those with mucocutaneous involvement (P < 0.01) and healthy controls (P = 0.001), and in male patients than in female patients (P < 0.001). There was no significant difference in homocysteine levels between the BD patients with mucocutaneous involvement and healthy subjects. In multiple regression analysis, serum homocysteine level was independently associated with thrombosis (odds ratio 1.29, P < 0.01), but male sex was not. CONCLUSIONS: This preliminary study suggests that elevated serum homocysteine levels may play some role in the development of venous and arterial thromboses in BD.

Kosar F, Sahin I, Gullu H, Cehreli S. International Journal of Cardiology. 99(2):355-7, 2005.

Behcet's disease (BD) is an inflammatory disorder of unknown origin, which usually presents with mucocutaneous, ocular, articular, vascular, gastrointestinal and central nervous system manifestations. Although cardiac involvement is not infrequent as a manifestation of Behcet's disease, coronary arteritis is very rarely reported. We suggest that the diagnosis of coronary arteritis should be considered in patients presenting acute myocardial infarction especially in young patients as underlying cause.

Association of aneurysmal and occlusive lesions in Behcet's disease.

Goueffic Y, Pistorius MA, Heymann MF, Chaillou P, Patra P.Annals of Vascular Surgery. 19(2):276-9, 2005.

Arterial involvement in Behcet's disease is rare, occurring in various locations with multiple clinical expressions. When Behcet's disease is associated with large arteries, lesions are usually in the form of aneurysms or occlusions. The simultaneous occurrence of these two lesions is even more unusual. We present a case of Behcet's disease in which arterial involvement included an iliac artery thrombosis and an asymptomatic aneurysm of the infrarenal abdominal aorta. Behcet's disease must be considered in the diagnosis of any unexplained inflammatory arteriopathy. Surgery is indicated for the majority of aneurysms and severe symptoms. The postoperative follow-up is based on noninvasive radiologic examinations.

Yekeler E, Tunaci A, Tunaci M, Kamali S, Gul A, Acunas B. Australasian Radiology. 49(2):182-4, 2005.

Arterial manifestations of Behcet's disease consist of aneurysm formation, stenosis and occlusion. Aneurysms in Behcet's disease most commonly involve the pulmonary arteries and have been shown to resolve with medical treatment. However, this regression pattern with medical therapy has not been reported for aortic aneurysms to date. We present a 43-year-old man with bilateral abdominal aortic aneurysms resulting from Behcet's disease resolving with medical therapy.

Relapsing arterial aneurysms in juvenile Behcet's disease.

Antar KA, Keiser HD, Peeva E.

Clinical Rheumatology. 24(1):72-5, 2005 Feb.

Vascular involvement in Behcet's disease has a wide range of clinical manifestations, the most common being thrombophlebitis and arterial aneurysms. Every major vessel may be involved, but aneurysms of the carotid artery are relatively rare in adults and even rarer in children. We describe here a case of a 13-year-old boy with Behcet's disease with a recurrent carotid artery aneurysm treated with arterial ligation and immunosuppressive therapy who later developed an abdominal aortic aneurysm in the absence of other indications of disease activity. This case underscores the need for continuous monitoring of patients with vascular manifestations of Behcet's disease.

Right ventricular outflow tract thrombus in a case of Behcet's disease.

Ozer N, Ciftci O, Demirci M, Atalar E, Ovunc K, Aksoyek S, Ozmen F, Kes S. Journal of the American Society of Echocardiography. 18(4):380-2, 2005.

Behcet's disease is known as a chronic systemic vasculitic syndrome, the hallmark of which is recurrent oral aphthous and genital ulcerations and uveitis. Vascular involvement, mainly thrombosis, reportedly affects as many as one-third of patients. Cardiac involvement, however, is very rare. We present in this report a young female patient developing right ventricular thrombus while being treated medically.

Castelli P, Caronno R, Piffaretti G, Tozzi M, Lomazzi C, Lagana D, Carrafiello G, Cuffari S. Journal of Vascular Surgery. 41(3):548-51, 2005.

Behcet disease is a heterogeneous, multisystem inflammatory disorder; vascular involvement has been considered to result from systemic vasculitis, which most frequently affects veins and occurs in 5% to 10% of these patients. However, superior vena cava (SVC) involvement is rare; it accounts for only 6% of the cases. The aim of this article is to a report on a case of SVC recanalization through the use of fibrinolysis and self-expanding stents as treatment of life-threatening SVC syndrome in a young male patient with Behcet disease.

Normayah K, Mazri YM, Suib I, Zainal AA. Medical Journal of Malaysia. 59(4):547-9, 2004.

A young gentleman presented with a history of multiple pseudoaneurysms of the right carotid artery, left radial artery, right femoral artery as well as deep vein thrombosis involving the right femoral vein. A diagnosis of Behcet's disease was made on the basis of his history of recurrent oral and genital ulceration with characteristic eye lesion.

Nayak RH, Dhas L, Mullasari AS. Journal of Invasive Cardiology. 11(9):575-6, 1999.

We describe a patient with Behcet's syndrome who had peripheral vascular disease involving the left subclavian artery for which angioplasty with stent placement was performed and reangioplasty done for in-stent restenosis. She presented with recurrence one year after stent placement; angiography revealed diffuse disease of the axillary and radial arteries with mild to moderate restenosis at the site of stent placement.

Feki M, Houman H, Ghannouchi M, Smiti-Khanfir M, Hamzaoui K, Matri LE, Mebazaa A, Kaabachi N.Clinical Chemistry & Laboratory Medicine. 42(12):1417-23, 2004.

Plasma homocysteine was assessed in Behcet's disease (BD) patients in order to determine the prevalence of hyperhomocysteinaemia in BD and to test its association with clinical manifestations of the disease. The study included 59 patients with BD and 118 age- and sex-matched healthy subjects. Plasma homocysteine, vitamin B(12) and folate were assessed by automated immunoassay methods. Hyperhomo-cysteinaemia was defined as plasma homocysteine >15 micromol/l. Plasma homocysteine concentrations and the prevalence of hyperhomocysteinaemia were significantly higher in BD patients than in controls [median (5th-95th percentile), 11.3 (6.6-28.1) vs. 10.6 (6.6-17.1) micromol/l, and 25.4% vs. 9.3%, respectively]. In BD patients, hyperhomocysteinaemia was related to male gender, disease severity and uveitis [odds ratio (OR), 5.32; 95% confidence interval (CI), 1.43-21.61; p = 0.008], but not to age, smoking, disease activity, deep venous thrombosis, arthritis or neurological involvement. The association between uveitis and hyperhomocysteinaemia persisted (multi-adjusted OR, 7.46; 95% CI, 1.03-54.3; p = 0.05) after adjusting for gender, age, disease activity and duration, smoking, deep venous thrombosis, and serum concentrations of creatinine, vitamin B(12) and folate. Plasma homocysteine should be measured in patients with BD, and the effect of B-vitamin supplementation should be tested in those with hyperhomo-cysteinaemia.

Elsharawy MA, Hassan KA, Al-Awami M, Al-Mulhim FA. Saudi Medical Journal. 25(12):2013-5, 2004.

Vascular involvement in Behcet's disease is rare approximately 14% venous and 1.6% arterial, serious and recurrent. We report a case of Behcet's disease with deep venous thrombosis and right iliac pseudoaneurysm which was repaired with polytetrafluoroethylene PTFE graft. The patient received warfarin, aspirin, clopidogrel, immunosuppressive and corticosteroids. Two months later the patient developed manifestations of superior vena cava thrombosis and the graft was blocked. Three months later, ischemia of the right foot deteriorated and left femoral artery to right femoral artery crossover PTFE graft was performed.

Caballol N, Dominguez A, Vidaller A, Martinez-Yelamos S. Neurologia 2005; 20:370-373

Arterial aneurysm formation is a rare manifestation in Behcet's disease (BD). Aneurysm rupture is the main cause of death among BD patients.
In recent years, endovascular treatment has been proposed as an effective and less invasive procedure compared with classical surgical treatment.
We report the case of a 29-year-old man with BD who had two syncopes precipitated by pressure on a left cervical mass and hemoptysis. Cerebral magnetic resonance imaging (MRI) showed acute infarction of left basal ganglia. Other examinations demonstrated carotid and pulmonary aneurysms that were treated by endovascular methods. The patient received treatment with corticosteroids and immunosuppressive therapy. First of all, the pulmonary aneurysm was treated by means of coil embolization. After anticoagulant therapy was initiated, a covered stent was placed to exclude the carotid aneurysm at the level of extracranial internal carotid artery (ICA), with no immediate complications. After twenty-seven months of follow-up the patient remains asymptomatic. Endovascular treatment may be a definite therapeutic option for extracranial carotid aneurysms in BD, although longer term follow-up studies are needed.

Atzeni F, Sarzi-Puttini P, Doria A, Boiardi L, Pipitone N, Salvarani C. Lupus 2005; 14:723-726

Behcet's disease (BD) is a multisystem disease of unknown etiology characterized by chronic relapsing orogenital ulcers, uveitis and systemic involvement including articular, gastrointestinal, cardiopulmonary, neurological and vascular pathology. The incidence and nature of cardiac involvement are not clearly elucidated. Cardiovascular manifestations have been reported in 7-46% of patients and mortality occurs in up to 20% of those patients with marked vascular involvement. Sporadic cases of endocarditis, myocarditis, pericarditis, acute myocardial infarction, aortic aneurysm, ventricular thrombosis, congestive cardiomyopathy and valvular dysfunction have been reported. This review discusses the general aspects of the pathogenic mechanisms and clinical features cardiovascular involvement in BD, and provides the data of cardiovascular involvement in a cohort of Italian BD patients.

Watanabe H, Oda H, Yoshida TS, Yamaura M, Takahashi K, Miida T, Takahashi N, Nakazawa S, Kanazawa H, Higuma N, Aizawa Y. International Heart Journal 2005; 46745-749.

Arterial aneurysms represent a severe complication of Behcet's disease. A 42-year-old woman with Behcet's disease had a recurrence of an aneurysm after two surgical repair attempts using grafts. A covered stent-graft was implanted in her iliac external artery to occlude the neck of the aneurysm at the anastomosis of the bypass graft to her external iliac artery. The procedure reduced the size of the aneurysm by allowing the formation of a thrombus within its cavity. The implantation of an endovascular stent-graft may be a sound alternative to surgical repair for aneurysms associated with Behcet's disease.

Szalat A, Durst R, Cohen A, Lotan C. Catheterization and Cardiovascular Interventions 2005; 66:203-208

Coronary artery aneurysm is an uncommon occurrence, yet it is described more often today than in the past as coronary angiography is now routinely used for diagnosis and treatment of ischemic heart disease. However, there is no therapeutic consensus regarding this finding. We present a case of giant coronary artery aneurysm and review the literature on the use of polytetrafluoroethylene-covered stents as a therapeutic option for this condition. Combined antiaggregant therapy is needed after the procedure. Randomized controlled trials of surgery versus covered stents are necessary to define the best treatment for large coronary artery aneurysms.

Current concepts in the etiology and treatment of Behcet disease. [Review]

Evereklioglu C. Survey of Ophthalmology. 50(4):297-350, 2005.

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. [References: 515]

Wu JJ, Huang DB, Pang KR, Hsu S, Tyring SK. British Journal of Dermatology. 153(2):254-73, 2005.

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. [References: 153]

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Gur-Toy G, Lenk N, Yalcin B, Aksaray S, Alli N. International Journal of Dermatology. 44(8):657-60, 2005.

BACKGROUND: Immune dysregulation has been shown to be one of the major aspects of the yet unknown pathogenesis of Behcet's disease. Interleukin-8 (IL-8), a major chemokine with pivotal effects concerning leukocytes and endothelial cells, has been found to be elevated in patients with Behcet's disease. AIM: To evaluate the significance of elevated levels of IL-8 with respect to the activity of Behcet's disease. METHODS: Sixty-seven consecutive patients with Behcet's disease (37 males, 30 females; 32.5 +/- 9.3 years) were enrolled in our study. The number of active clinical manifestations at the time of serum sampling was recorded. The degree of association between disease activity and IL-8, C-reactive protein, and erythrocyte sedimentation rate was assessed. RESULTS: Serum levels of IL-8 increased as the number of clinically involved organs increased (P < 0.05). C-reactive protein and the erythrocyte sedimentation rate showed no correlation with disease activity. CONCLUSIONS: Our study confirms that the IL-8 level is a more sensitive marker of disease activity than the erythrocyte sedimentation rate and C-reactive protein. It may be assumed that IL-8 plays an important role in the pathophysiology of Behcet's disease.

Korkmaz C, Ozcan A, Akcar N. Clinical & Experimental Rheumatology. 23(3):389-92, 2005.

OBJECTIVE: To determine the frequency of hypercalciuria and renal stones in ankylosing spondylitis (AS) sufferers. METHODS: This study involved 83 consecutive AS patients (21 female, 62 male; mean age 36.7 yr), 72 consecutive Behcet's disease (BD) patients (29 female, 43 male; mean age 37.7 yr) as disease control and 92 healthy control (HC) (26 female, 66 male; mean age 32.9 yr.) Twenty-four hour urine analyses for urinary calcium and uric acid levels were performed in each patient. Likewise, blood samples for erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), parathyroid hormone (PTH), calcium and uric acid evaluation were taken. Renal ultrasonography to evaluate the presence of renal stone was performed in patients with AS, as well as in the BD patients and HC individuals after a fasting period of 12 hours. RESULTS: 20 of the 80 (25%) patients with AS were diagnosed with renal stones. Only 4/72 (5.5%) BD patients, and 3/98 (3.3%) HC individuals had renal stones by ultrasonography. AS patients had a significantly higher frequency of renal stones compared with BD (p < 0.001) and HC (p < 0.0001). AS patients with renal stones were much older and their disease duration was much longer in comparison with AS patients without renal stones. Ultrasonographic and laboratory findings did not correlate. The number of AS patients with hypercalciuria who had renal stones was higher than that of AS patients who did not have renal stones (p < 0.01). There was a negative correlation between acute phase response and spinal mobility. CONCLUSION: Renal stone prevalence was found to increase in AS patients. The likelihood of renal stone formation was also found to increase with the extension of the disease duration of AS sufferers.

Kalantzis N, Papanikolaou IS, Giannakoulopoulou E, Alogari A, Kalantzis C, Papacharalampous X, Gabriel P, Alexandrakis G, Apostolopoulos P. Hepato-Gastroenterology. 52(62):414-9, 2005.

BACKGROUND/AIMS: This study presents our cumulative clinical experience from the use of wireless capsule endoscopy (WCE) in a large series of Greek patients with suspected small bowel (SB) diseases. METHODOLOGY: Over an 18-month period, 193 patients were evaluated with WCE for suspected SB diseases [108 obscure gastrointestinal (GI) bleeding, 32 chronic diarrhea, 22 suspected and 6 diagnosed Crohn's disease, 16 abdominal pain, 4 refractory celiac disease, 3 polyposis syndromes, 1 Rendu-Osler-Weber disease and 1 Behcet disease]. All patients had undergone upper GI endoscopy and total colonoscopy. WCE findings were characterized as specific or non-specific, depending on whether the patient's signs and symptoms could be sufficiently attributed to them or not. RESULTS: One or more abnormal findings were detected in 161/193 (83%) patients; these were classified as specific in 91/193 (47%). The diagnostic yield of WCE (in terms of specific findings) was significantly higher when evaluating patients with obscure GI bleeding, compared to chronic diarrhea (52% vs. 25%, respectively, p=0.013, 95% CI: 1.33-7.83). Among obscure GI bleeders, specific findings were significantly more in the group of overt rather than occult bleeders (66% vs. 42% respectively, p=0.026, 95% CI: 1.19-5.88). In patients referred for diagnosed or suspected Crohn's disease, WCE findings were compatible with the diagnosis in 5/6 cases (83%) and in 8/22 cases (36%), respectively. In 9 more patients from other groups with a negative previous diagnostic work-up, WCE helped in diagnosing Crohn's disease. Among 16 patients evaluated for abdominal pain, specific findings were identified only in 1 (6%). CONCLUSIONS: WCE seems to be a very useful diagnostic tool in the evaluation of GI bleeding of obscure origin (in both overt and occult bleeders), as well as suspected Crohn's disease. In other indications, such as unexplained chronic diarrhea and abdominal pain, it completes the traditional work-up, but abnormal findings need to be better delineated before WCE can be widely recommended in these cases.

Dervis E, Geyik N. Journal of Dermatology. 32(4):266-72, 2005.

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.

Fibromyalgia in Iraqi patients with Behcet's disease.

Al-Izzi MK, Jabber AS. Journal Medical Libanais - Lebanese Medical Journal. 52(2):86-90, 2004.

OBJECTIVE: To determine the prevalence of fibromyalgia syndrome (FMS) in Iraqi patients with Behcet's disease. METHODS: The two-stage classification process proposed by the 1990 American College of Rheumatology (ACR) multicenter criteria committee on fibromyalgia was applied after completion of a questionnaire about Behcet's disease clinical features. Stage I: A plain questionnaire to a sample of 90 (41 female and 49 male) patients with Behcet's disease, mean age 37.5 years. Stage II: All patients with wide spread pain were examined by a physician blinded to the diagnosis of Behcet's disease for 18 tender points and 4 control non-tender points. A sample of 40 (16 female and 24 male) healthy individuals were examined as controls. RESULTS: There were 53 (58.9%) patients with BD with widespread pain compared to 6 (15%) individuals in the control group, whereas only 8 (8.9%) (7 female and 1 male) patients with BD fulfilled the 1990 ACR criteria for classification of FMS, compared to one individual in the controls (2.5%). There were significantly more females with BD having FMS compared to male patients. Also the FMS associated features were more prevalent in BD group compared to control group. CONCLUSIONS: FMS and widespread pain are more prevalent in patients with BD than in the general population while it is less prevalent when compared with other connective tissue diseases. There is a trend for an increased frequency of FMS in females with BD. BD patients with FMS may benefit from psychological evaluation as a part of their treatment.

Ning-Sheng L, Chun-Liang L, Ray-Sheng L. Scandinavian Journal of Rheumatology. 33(6):437-40, 2004.

A 55-year-old male presented a productive cough, haemoptysis, and intermittent fever for 2 weeks. He was diagnosed as having Behcet's disease (BD) with manifestations of recurrent oral ulcers, genital ulcers, erythema nodosum, a gastro/duodenal ulcer, colon ulcers, and folliculitis. After admission, a chest X-ray showed bilateral ill-defined patches. Subsequent chest computerized tomography revealed multiple patchy consolidations over the left and right lower lobes. Because of poor response to oxacillin antibiotic treatment, an open lung biopsy was arranged. Bronchiolitis obliterans organizing pneumonia (BOOP) was diagnosed. Methylprednisolone pulse therapy, followed by prednisolone and cyclophosphamide maintenance, was prescribed. The patient responded well, with clinical and radiological resolution. Early diagnosis with proper treatment is crucial in the management of this lethal pulmonary disease.

Behcet's disease: a review. [Review]

Al-Otaibi LM. Porter SR. Poate TW Journal of Dental Research. 84(3):209-22, 2005.

Behcet's disease (BD) is a multi-system inflammatory disorder dominated clinically by recurrent oral and genital ulceration, uveitis, and erythema nodosum. Behcet's disease runs a chronic course, with unpredictable exacerbations and remissions whose frequency and severity may diminish with time. Behcet's disease typically arises in young adults, although childhood-onset BD has also been reported. The disease can affect both genders and has a worldwide distribution, although it is more prevalent in countries of the ancient Silk Route. The cause of BD remains unknown, although an autoimmune reaction triggered by an infectious agent in a genetically predisposed individual has been suggested. The treatment of BD is symptomatic and empirical, but generally specific to the clinical features of each patient. The majority of affected individuals do not have life-threatening disease, although mortality can be associated with vascular-thrombotic and neurological disease.

Mumcu G. Ergun T. Elbir Y. Eksioglu-Demiralp E. Yavuz S. Atalay T. Direskeneli H. Journal of Oral Pathology & Medicine. 34(1):13-6, 2005.

BACKGROUND: The aim of this study was to evaluate the effects of azithromycin on mucocutaneous manifestations, oral health and immune response in Behcet's disease (BD). METHODS: Eight BD patients with active mucocutaneous symptoms were treated with azithromycin for 4 weeks. Oral health, clinical manifestations and in vitro interleukin (IL)-12, interferon (IFN)-gamma, IL-10 and monocyte chemotactic protein (MCP)-1 responses were evaluated before and after treatment. RESULTS: The number of folliculitic lesions, healing time of oral ulcers and scores of plaque indexes (PLIs) were lower after azithromycin treatment (P<0.05). Scores of PLIs correlated positively with the healing time of oral ulcers (P=0.02). Although a trend towards increased stimulated IL-10 responses with azithromycin was observed, no statistically significant difference was found. Stimulated and unstimulated MCP-1, IFN-gamma and IL-12 responses were similar before and after treatment (P>0.05). CONCLUSION: Azithromycin was observed to be effective in decreasing folliculitic lesions and fastening the healing time of oral ulcers in BD.

Coskun B. Ozturk P. Saral Y. International Journal of Clinical Practice. 59(1):69-71, 2005.

Erythema nodosum (EN)-like lesions and superficial thrombophlebitis (ST) are skin symptoms that are frequently observed in Behcet's disease. In most of the patients, skin symptoms precede serious visceral involvement. The study included 78 patients whose mean age was 33.48 +/- 6.16 (21-48). Of the cases, 37 were females and 41 were males. Forty-two patients (53.8%) had EN-like lesion and 12 (15.3%) had ST. Six of 54 patients, who had EN-like lesion and ST, were found to have no visceral involvement. In six of the remaining 48 patients, it was established that visceral involvement developed before lesions. It was found in 42 patients that these two lesions preceded visceral involvement. In addition, 18 of 24 patients who did not have these two lesions, do not still have visceral involvement. Therefore, we think that EN-like lesion and ST can be guiding in predicting serious visceral involvement and complications that can develop in advanced stages of Behcet's disease.

Bicer A. Tursen U. Kaya TI. Ozer C. Camdeviren H. Ikizoglu G. Erdogan C

Rheumatology International. 24(6):355-8, 2004.

Behcet's disease is a complex, multisystemic, inflammatory disorder characterized clinically by recurrent oral and genital ulcerations as well as uveitis, sometimes leading to blindness. The etiology and pathogenesis of this syndrome remain obscure. However, various factors are suspected, including genetic propensity, infectious precipitants, and immunological abnormalities. Considering the chronicity and unclear etiology of the disease, we conducted a prospective investigation of a possible alteration in the bone mineral density of affected persons. Thirty-five patients (18 males and 17 females, mean age 38.02+/-7.93 years) diagnosed with Behcet's disease and 33 sex- and age-matched healthy controls (14 males and 19 females, mean age 40.06+/-7.66 years) were seen on an outpatient basis, and bone densitometry measurements were done from June 2000 to December 2002 at the Mersin University Hospital in Turkey. Postmenopausal women with Behcet's disease and patients receiving oral corticosteroid therapy were excluded from the study. The mean disease duration was 6.68+/-7.05 years. Bone mineral density was measured with dual X-ray absorptiometry at the lumbar spine and right femur. The mean Z scores of the patient and control groups were -0.50+/-1.06 and -0.13+/-0.92 at the lumbar spine, respectively, and 0.38+/-1.07 and 0.45+/-1.20 at the right femur, respectively. No significant differences in bone mineral density values were detected in the groups at either the lumbar (P = 0.15) or right femur (P = 0.82) site. Body mass index and disease duration did not influence bone mineral density, and age had a positive correlation with bone mineral density in patients with Behcet's disease. In conclusion, although it is difficult to draw definite conclusions due to the relatively small sample size, our study confirms that bone mineral density in Behcet's disease was not lower than in healthy subjects.

Koc B. Aymelek S. Sonmez A. Yilmaz MI. Kocar H. Rheumatology International. 24(6):347-50, 2004.

Behcet's disease (BD) is a chronic, relapsing, systemic vasculitis with unknown etiology. During the progression of the disease, gastrointestinal involvement can be observed. The aim of this study was to find out the predictive value of the sucrose permeability test in detecting gastrointestinal mucosal damage in BD. Twenty-six male Behcet's patients and 21 age- and sex-matched controls were enrolled in the study. Seventeen patients had active disease, while nine did not. Active disease was defined as having elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels or at least two of the diagnostic criteria in the previous month. Patients and controls were investigated with the upper gastrointestinal permeability test. Of all the patients, 25 were investigated endoscopically. The urine sucrose levels were significantly higher in patients than in the control group (P = 0.0001) and in patients with active disease than those with inactive disease (P < 0.0001), while urine sucrose of patients with inactive disease and the control group did not differ. The endoscopic findings were not specific to BD. Active and inactive BD had similar endoscopic findings. Increased upper gastrointestinal permeability was established in patients with BD. This increased permeability was not related to a specific gastrointestinal BD lesion. Further studies with larger series must be performed in order to determine the value of the sucrose permeability test in detecting mucosal involvement in BD.

Yigit O. Alkan S. Basak T. Dadas B. European Archives of Oto-Rhino-Laryngology. 262(2):151-3, 2005.

A 40-year-old man presented with a single, major ulcer in the hypopharynx. Histopathological examination revealed necrosis and extensive vasculitis. Since the patient had recurrent oral ulcers and genital ulcers in his history and a positive pathergy test, a diagnosis of Behcet's disease was made, according to the 1992 international criteria. Behcet's disease should be kept in mind in differential diagnoses of hypopharyngeal ulcerous lesions, and detailed case histories should be taken.

Blood-tinged stools and blood-shot eyes.

Lee JJ. Beebout S. Lee AG. Tuetken R. Weinberg DA. Survey of Ophthalmology. 50(5):476-84, 2005.

A 37-year-old woman developed red painful eyes, diarrhea, and painful oral and genital ulcerations. Optic disk swelling was noted bilaterally. Colonoscopy was significant for granulomatous lesions consistent with Crohn disease. Epidemiologic factors, diagnostic criteria, and differentiating characteristics for Behcet disease and Crohn disease are discussed.

Gubler C. Bauerfeind P. Endoscopy. 37(7):689, 2005.

Hasegawa T. Kanno S. Kato M. Fujihara K. Shiga Y. Itoyama Y.

European Journal of Neurology. 12(8):661-2, 2005.

Comment on: J Vasc Surg. 2005;41(1):53-8; Journal of Vascular Surgery. 42(1):181; author reply 181-2, 2005.

Images in clinical medicine. Bilateral pulmonary-artery aneurysms in Behcet's syndrome.

Lohani S. Niven R. New England Journal of Medicine. 353(4):400, 2005.

Oguzhan A. Gul A. Asik R. Inanc T. Ozdogru I. Topsakal R. Eryol NK.

Anadolu Kardiyoloji Dergisi. 5(2):154, 2005.

Boyer JF, Jamard B, El Mahou S, Laroche M, Mazieres B, Cantagrel A, Constantin A. Clinical & Experimental Rheumatology. 23(2):274-5, 2005.

Merkel-cell carcinoma in Behcet's disease.

Satolli F, Venturi C, Vescovi V, Morrone P, De Panfilis G. Acta Dermato-Venereologica. 85(1):79, 2005.

Aroussi AA, Redai M, El Ouardi F, Mehadji BE. Journal of Thoracic & Cardiovascular Surgery. 129(5):1170-1, 2005.

Bills G, Kaufman RH, Bornstein J, Haefner H. Journal of Lower Genital Tract Disease. 9(1):55-8, 2005.

Psoriasis associated with Behcet's disease.

Ujihara M, Kodama H. Journal of Dermatology. 32(2):147-9, 2005.

Trager JD. Journal of Pediatric & Adolescent Gynecology. 17(6):397-401, 2004.

Elevated FVIII and FIX level in a Behcet's disease patient with intracardiac thrombosis and pulmonary arterial aneurysms. [Review]

Atalay F, Ernam D, Okten F, Akar N. Thrombosis Research. 115(1-2):159-61, 2005.

Oguz AK. Ozcakar L. Kaymak B. Rheumatology. 44(3):401, 2005.

Dolar E, Uslusoy H, Kiyici M, Gurel S, Nak SG, Gulten M, Zorluoglu A, Saricaoglu H, Memik F. Rheumatology 2005; 44:1327-8