Differences in clinical manifestations, treatment, and concordance rates with two major sets of criteria for Beh‡et's syndrome for patients in the US and Japan: data from a large, three-center cohort study

To compare Beh double dagger et'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. 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. 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. 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.