Clinical Profile of Vascular Behcets Disease Patients Over a 30-year Period: Insights from an Iranian Retrospective Cohort Study

Vascular manifestations are rare and cause morbidity and mortality in patients with Behcets disease (BD). Owing to the lack of evidence, we aimed to retrospectively evaluate the clinical features and associated risk factors of vascular BD. This retrospective cohort study was conducted at the Rheumatology Research Center, Tehran, Iran, from 1982 to 2018. The demographics, laboratory results, severity at diagnosis, diagnosis interval, and clinical manifestations divided into major events as well as sub-events at each visit were recorded. Of 57 patients, 43 (75.44%) were male, with an M:F ratio of 3.07. The mean age at onset was 32.00 {+/-} 8.05 years. The three major clinical events repeated during the study period were mucocutaneous (41.30%), ocular (17%), and vascular (16.8%). DVT (33.3 %), SVT (21.84 %), and thrombosis of the large vein extremities (17.24 %) were the most common vascular events. The presence of HLA-B51 was found to have a statistically significant impact on the distinct number of events (B=-0.39, 95% CI: -0.74, -0.03, p=0.039). Severity at diagnosis (Moderate Vs. mild and Severe Vs. mild) were found to be statistically significant predictors of the logarithm of distinct number of events (B=-0.68, 95% CI: -1.13, -0.24, p=0.004; B=-0.51, 95% CI: -0.93, -0.10, p=0.020). The odds of developing vasculitis decreased faster in patients with a family history of OA than in those without a family history of OA. The other variables were not found to have a statistically significant impact on the logarithm of distinct number of events. A family history of OA, HLA- B51 positivity, and disease severity at diagnosis were associated with the occurrence of vascular BD. Further longitudinal and large-sample size studies should be conducted to evaluate the risk factors for vascular events.