Clinical characteristics of Behçet's syndrome in Shanghai database: Baseline data of a cross-sectional cohort study

Objective: Behçet's syndrome (BS) is a variant vessel vasculitis that can involve multiple organs, with highly heterogeneous clinical manifestations. This study aims to analyze baseline data of BS patients to enhance the comprehension of its clinical features.

Methods: This study included 1216 registered cases of BS patients referred to Huadong Hospital affiliated with Fudan University. Each patient was thoroughly assessed and recorded for demographic data, clinical manifestations, gastrointestinal endoscope, imaging, etc. RESULTS: Significant gender differences were observed in clinical manifestations. Pseudofolliculitis (p < .001), uveitis (p = .003), vascular (p < .001), and cardiovascular involvement (p < .001) were significantly more prevalent in male BS patients, while genital ulcers (p = .011) and erythema nodosum (p = .009) were more common among the female. Furthermore, pseudofolliculitis (44.3%, 37.4% vs. 25.0%, p < .001), pathergy test positivity (37.0%, 24.5% vs. 12.6%, p < .001), and uveitis (18.8%, 18.4% vs. 11.2%, p < .001) showed higher incidence rates in the 16-35 years age group. Vascular involvement (11.1%, 18.0% vs. 15.8%, p < .001) notably increased in the 36-50 years age group. Additionally, the ISG diagnostic criteria were more likely to be met in the 16-35 age group (OR: 2.039, 95% CI: 1.581-2.631, p < .001), whereas the ICBD criteria were less likely to be met in the 16-35 age group (OR: 0.266, 95% CI: 0.150-0.474, p < .001).

Conclusions: This study provided data on the baseline of clinical features of BS in a single center, BS patients presented significant heterogeneity, showing different manifestations across various genders and age groups. This diversity might contribute to a better understanding of BS clinical features and pave the way for future multi-center studies.