Behçet's disease in southern Chinese patients

Abstract

Objective. To describe the clinical characteristics of Behçet's disease (BD) in a southern Chinese population and compare them with those reported in other ethnic groups. Methods. Patients with BD from 4 large regional hospitals in Hong Kong were identified from the hospital information retrieval system. Their records were retrospectively reviewed and the diagnosis was verified by at least 2 rheumatologists using the International Study Group (ISG) criteria. Demographic data and various clinical features of our patients were collected and analyzed. Results. Between 1978 and 2000, 37 adult patients with definite BD according to the ISG criteria were identified. All patients were ethnic southern Chinese. There were 19 women and 18 men (F:M = 1.1: 1). The mean age at diagnosis was 36.2 ± 10 (18-74) years. Presenting features at diagnosis, in decreasing order of frequency, were oral ulceration (100%), genital ulceration (81%), skin lesions (73%), arthritis/arthralgia (54%), and ocular lesions (35%). Among skin lesions, the commonest manifestations were erythema nodosum (74%), folliculitis (44%), and acneiform nodules (30%). Arthritis was usually polyarticular (75%) and symmetrical, and involved the upper limb joints. Anterior uveitis was the most common ocular complication (77%) and was often bilateral. Involvement of other systems such as the central nervous system, gastrointestinal (GI) tract, and cardiopulmonary system was uncommon. Vascular complications were rare. Only 2 (6%) patients had a positive pathergy test. Our patients were followed for a mean of 59.9 ± 52 months. Apart from recurrent attacks of orogenital ulceration and uveitis, new systemic features were unusual. One patient died of bowel perforation as a result of active GI disease. Compared with other reported series, our patients had fewer ocular and systemic manifestations, and a significantly lower incidence of the pathergy reaction. Conclusions. BD is rare among southern Chinese in Hong Kong and tends to run a more benign course with less systemic involvement and fewer serious ocular complications. Whether the observed difference in clinical manifestations of the disease is related to a different HLA association or environmental factors warrants further studies.