Clinical features of early-stage possible Behçet’s disease patients with a variant-type major organ involvement in Japan

AbstractBackground: Clinical data of patients with entro-, vasculo-, and neuro-variant possible Behcet’s disease (BD) based on Japanese criteria has not yet comprehensively reported.Methods: This ongoing nation-wide registration has been carried out by the Japanese Ministry of Health, Labour and Welfare. The Ministry asked physicians who diagnosed a patient with confirmed or possible BD to register the patient data by filling out a registration form. The Ministry provided us with the dataset after unlinkable anonymization. We analyzed 2003–2014 database generated from the early stage new cases.Results: Among the 7950 analyzable cases, 694 (8.7%) had variant-type possible BD without satisfying complete/incomplete criteria. Of the 694 patients, 479, 46, and 169 had entero-, vasculo-, and neuro-variant possible BD, respectively. Out of these 694 patients, 35 (5.0%) and 154 (22.2%) satisfied the International Study Group criteria and the International Criteria of BD, respectively. Entero-variant possible pati...