Cardiac involvement in Behçet's syndrome: findings from a clinically driven cardiological evaluation

To characterize prevalence, patterns, and clinical correlates of cardiac involvement in a cohort of patients with Behçet's syndrome (BS) undergoing clinically driven cardiological assessment.

This retrospective study included adult patients with BS, followed at a tertiary referral centre (Florence, Italy), who underwent clinically driven cardiological evaluation. Clinical, demographic and therapeutic characteristics were compared in patients with and without evidence of cardiac involvement. Timing, type of cardiac involvement, disease activity, cardiovascular risk factors and treatments were analysed.

Among 312 patients with BS, 90 underwent cardiological assessment and 49 had confirmed cardiac involvement. Arrhythmias were the most frequent manifestation (n = 15), followed by pericarditis (n = 12) and ischaemic heart disease (n = 8). Cardiac involvement occurred a median of 6.1 (2.5-8.7) years after BS diagnosis, although in a proportion of patients it preceded or coincided with diagnosis. Most patients had active systemic disease at the time of the cardiac involvement (93.9%) and were receiving immunosuppressant treatment (84%). Over 80% had at least one traditional cardiovascular risk factor, yet <40% were receiving cardiovascular primary prevention therapy before the first event. Male patients more frequently experienced ischaemic manifestations, while arrhythmias were more common in females. No cardiac-related deaths were observed.

Cardiac involvement in BS is more common than traditionally reported when actively investigated. Cardiac involvement frequently occurs during active disease and in patients with a high burden of cardiovascular risk factors but suboptimal preventive therapy. These findings support the need for integrated cardio-rheumatologic management in BS.