Clinical and demographic features of Behçets: a retrospective analysis of 4007 patients from a single centre in Turkey.

Abstract

Background: Behçets is a multisystem inflammatory condition that can lead to severe vascular and neurological complications.

Objectives: To evaluate the demographic and clinical characteristics of a large single-centre cohort of patients with Behçets and to investigate the associations of sex, age at onset and human leucocyte antigen B51 (HLA-B51) status with organ involvement patterns.

Methods: A retrospective cross-sectional study was conducted on 4007 patients who had been followed-up since 2010 at the Behçet's Disease Research and Application Center at Istanbul University-Cerrahpaşa, Turkey. Demographic data and details of clinical features and organ involvement were extracted and analysed statistically based on sex, age of disease onset and HLA-B51 status.

Results: The mean (SD) age of patients was 42.9 (11.4) years, with a male to female ratio of 1.1:1. Men exhibited higher rates of ocular, vascular and neurological involvement, while women had more joint involvement. HLA-B51 was associated with joint and ocular involvement and pathergy positivity. Early-onset disease (onset < 16 years) was observed in 11% (n = 448/4007) of patients and was associated with increased rates of systemic involvement. Joint involvement (n = 1553/4007, 39%) mostly appeared as arthralgia or arthritis. Gastrointestinal involvement (n = 115/4007, 2.9%) was associated with diabetes mellitus. Pathergy positivity was observed in 21% (n = 823/4007) of patients and was associated with early disease onset and smoking. Multivariable analyses showed that male sex and early disease onset were associated with ocular and neurological involvement, whereas vascular involvement was associated with male sex, early onset and smoking.

Conclusions: Organ involvement in Behçets varies by sex, age at onset and HLA-B51 status, highlighting the importance of these factors in clinical evaluation.