Clinical and Demographical Characteristics of Familial Behçet’s Disease (Southeast Marmara Region)

Abstract

Background: Familial aggregation in Behçet’s disease (BD) has been reported in Turkish and Japanese populations. While the frequency of familial cases has been reported to be 2–5% worldwide, this rate reaches up to 15% in the Middle East. Objective: This study aimed to determine the incidence of familial BD cases followed in the BD polyclinic and to compare their clinical and demographic characteristics to those observed in sporadic cases. Methods: Data related to BD patients who were followed between 1995 and 2014 were collected from computerized archive records and were assessed for detailed family histories. Only first-degree relatives (brother, sister, mother, father, children) were considered to be cases of familial BD. Clinical and demographic features were retrieved. Our BD polyclinic is located in the Southeast Marmara Region in Turkey. Results: BD was detected in 36 first-degree relatives of 33 patients out of 840 patients with BD. A total of 45 patients were diagnosed as familial BD;23 were female, and 22 were male. In our patients, the incidence of familial BD was determined to be 3.9%. The rates for HLA-B5 positivity, ocular involvement, genital ulcers, and erythema nodosum were determined to be 86.6% (26/30), 26.6%, 82.2%, and 60%, respectively. None of the patients had neurological involvement, but 2 had vascular involvement. Conclusion: This study may contribute to the epidemiological data of BD from Turkey.