Clinical features of cardiac and vascular involvements in Behçet's syndrome: a cross-sectional study from Shanghai Behçet's syndrome database.

OBJECTIVES

Cardiovascular involvement is an important cause of morbidity and mortality in patients with Behçet's syndrome (BS). This study aimed to investigate the clinical characteristics and laboratory findings of patients with cardiovascular BS.

METHODS

This is a cross-sectional study of BS patients who were hospitalized in Huadong Hospital Affiliated to Fudan University. The characteristics of cardiovascular BS were described, and the similarities and differences between cardiac BS and vascular BS were analyzed by two-tailed Student's test and Pearson's chi-square test.

RESULTS

A total of 2466 patients with BS were included in this study. Cardiovascular BS accounted for 12.8% (316 cases), including 144 cases of cardiac BS, 123 cases of vascular BS, and 49 cases of cardiac BS coexisting with vascular BS. Smoking history (P < 0.001), drinking history (P < 0.001), folliculitis (P = 0.015), and arthralgia (P = 0.015) were more common in the cardiac BS. Contrarily, thrombophlebitis (P = 0.009), CNS involvement (P = 0.028), and cerebral aneurysm (P = 0.020) were more common in the vascular BS. Compared with vascular BS, cardiac BS had higher levels of inflammatory markers, blood lipids, and uric acid and was more likely to have coagulopathy. Aortic regurgitation (66.0%) was the most common manifestation of cardiac BS. Cardiac valve replacement was performed in 69 cases (46.5%). However, 48 cases had occurred perivalvular leakage and required the Bentall procedure as remedial measures. Among vascular BS, the proportion of patients with deep vein thrombosis of the lower extremities (45.5%) was the largest, followed by the involvement of the superior vena cava (19.5%) and brachiocephalic artery (11.4%).

CONCLUSION

When it is observed that BS patients have a history of drinking, hyperuricemia, and elevated lactate dehydrogenase, the possibility of cardiac involvement should be considered. Severe valve regurgitation requires surgical treatment, and the Bentall procedure should be the first choice. Key Points • In this study, we describe the characteristics and risk factors of cardiac and vascular BS and explore the similarities and differences between these two types.