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Background Behcet's syndrome (BS) is a systemic vasculitis that may different pathogenic mechanisms leading to different manifestations, such as eye, mucocutaneous and GI disease. We started a dedicated Behcet's clinic in 2004 and have treated over 850 patients to date. Objectives To determine the response to therapy depending on type of organ involvement and documentation of improvement with patient reported outcomes. Methods All patients seen at the Center complete MDHAQ, medical history, medication use, Behcet's specific history, ethnic and demographic information forms. In addition a Behcet Syndrome Activity Score (BSAS) is also completed by all Behcet patients. These data are prospectively collected and updated each visit. Patients were divided into eye disease only, GI only, both or none groups and compared for disease activity and medication, specifically azathioprine, use. They could have mucocutaneous disease in addition to above manifestations Results 484 patients (78% female, disease duration 4.8 (7.1) years, age 35.3 (13.8)) were analyzed. 244 patients had no eye or GI disease, 83 had eye, 111 GI and 46 both eye and GI involvement. Both groups of azathioprine treated and untreated patients showed improvement in their disease activity scores but the improvement were more pronounced for GI disease by BSAS. RAPID3 responses were more in the azathioprine treated group with eye and both eye and GI disease. Patients with no eye or GI disease did similarly with or without azathioprine. (Table presented) Conclusions In this cohort of 484 Behcet patients, some treatment response differences were noted between patients with eye or GI involvement vs those who did not have these. Overall, azathioprine, led to better outcomes regardless of organ involvement. It was possible to demonstrate these improvements using patient reported outcomes, RAPID3 and BSAS, as part of routine clinical care

Behcet's syndrome (BS) shows a peculiar distribution, with a much higher prevalence in countries along the ancient Silk Road compared with rest of the world. BS also seems to follow a more severe course in ethnic groups with higher prevalence. Diagnosis depends on clinical findings. Criteria sets may not help in patients with less frequent types of involvement. Management strategies should be modified according to the age and sex of the patient and the organs involved. Being a serious health problem in endemic areas, BS also attracts global attention as a model to study inflammatory diseases of unknown cause.

OBJECTIVE: To compare Behcet's syndrome (BS) cohorts from the US and Japan in terms of rates of concordance with the International Study Group (ISG) criteria and Japanese criteria, disease manifestations, and treatment. METHODS: All BS patients seen at the NYU Hospital for Joint Diseases in the US and the Kameda Medical Center and St. Luke's International Hospital in Japan between 2003 and 2010 were included. Diagnosis of BS was made on the basis of clinical manifestations and the clinical decisions of experienced specialists familiar with BS. We classified the patients into complete and incomplete types based on their symptoms; both complete or incomplete types were assumed to fulfil the Japanese criteria. RESULTS: A total of 769 patients (US n = 634, Japan n = 135) were reviewed. 61.5 % in the US and 63.7 % in Japan fulfilled the ISG criteria. Similarly, there was no difference in the proportions of US and Japanese patients who fulfilled the Japanese criteria. Japanese patients were less likely to be female and to have genital ulcers, but were more likely to have epididymitis and pulmonary disease. Significantly more patients were treated with colchicine, sulfasalazine/mesalazine, and NSAIDs in Japan, while significantly more patients in the US received first-line immunosuppressants. CONCLUSIONS: The concordance rates for ISG and Japanese criteria fulfillment in the US and Japan were not significantly different. These findings could help to clarify regional differences in the diagnostic and clinical features of BS.