Abstract
IntroductionBehcet’s syndrome (BS) is a complex, heterogeneous disorder. However, classification of its subgroups is still debated. The purpose of this study was to investigate the clinical features and aggregation of patients with BS in China, based on manifestations and organ involvements.MethodsThis was a cross-sectional study of BS patients in Huadong Hospital of Fudan University between September 2012 and January 2020. We calculated relative risks (RRs) of clinical variables according to sex. Moreover, we conducted a hierarchical cluster analysis applied according to eighteen variables to determine subgroups of patients.ResultsA total of 860 BS patients were included. Male sex was associated with ocular involvement (RR 2.32, 95% CI 1.67, 3.22, P < 0.0001), vascular involvement (RR 2.00, 95% CI 1.23, 3.23, P = 0.004), cardiac lesion (RR 5.46, 95% CI 2.33, 12.77, P < 0.0001), and central nervous system involvement (RR 2.95, 95% CI 1.07, 6.78, P = 0.007) and was negatively associated with genital ulcers (RR 0.84, 95% CI 0.79, 0.91, P < 0.0001). Five clusters (C1–C5) were observed. C1 (n = 307) showed the skin and mucosa type. In C2 (n = 124), all had articular involvement, barely having major organ involvement except for 18 cases with intestinal lesions. In C3 (n = 156), the gastrointestinal type, 144 patients presented with intestinal involvement, and 36 patients with esophageal ulcers. In C4 (n = 142), all subjects presented with uveitis. C5 (n = 131) consisted of 44 patients with cardiac lesions, 58 with vascular involvement, and 26 cases having central nervous system involvement.ConclusionOur analysis confirmed sex differences in phenotypes of BS. Cluster analysis identified gastrointestinal, uveitis, and cardiovascular involvement cluster separately in different subsets, which represents the most commonly involved organs. Further research is required to replicate and clarify the patterns of phenotype in BS.
Highlights
Behcet’s syndrome (BS) is a complex, heterogeneous disorder
Sex-phenotype analysis In regard to sex-associated clinical features (Table 1), male sex was correlated with ocular involvement [relative risks (RRs) 2.32], including panuveitis [2.72 (1.88, 3.92)]; vascular involvement [2.00 (1.23, 3.23)], including deep venous thrombosis [2.71 (1.17, 6.27)] and arterial thrombosis or aneurysms [1.97 (1.07, 3.64)]; cardiac lesions [5.46 (2.33, 12.77)]; and central nervous system involvement [2.73 (1.10, 6.76)]
We conducted detailed and comprehensive analyses in a large cohort of BS patients in China, from which we identified distinct clinical manifestations and major organ involvement patterns between male and female
Summary
The purpose of this study was to investigate the clinical features and aggregation of patients with BS in China, based on manifestations and organ involvements. The purpose of the present study is to identify sex-associated differences in manifestations and major organ involvements
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