Purpose of the Review: Behçet’s Syndrome (BS) is a multisystemic vasculitis that can affect the heart, leading to pericarditis, myocarditis, intracardiac thrombosis, endomyocardial fibrosis, valvular dysfunction, and coronary artery disease. This review summarizes the clinical presentation, diagnostic challenges, and therapeutic strategies for cardiac involvement in BS. Recent Findings: Advanced imaging techniques have revealed subclinical cardiac involvement in BS. Myocardial dysfunction and fibrosis contribute to heart failure and arrhythmias, while intracardiac thrombi often coexist with pulmonary artery involvement. Coronary artery vasculitis and aneurysms may mimic atherosclerotic disease, complicating diagnosis. Biologic therapies, including TNF-α inhibitors, show promise in refractory cases. Summary: Early diagnosis and immunosuppressive therapy are crucial. A multidisciplinary approach is essential to managing cardiac complications and optimizing patient outcomes. Future research should refine screening protocols and explore targeted immunotherapies for BS-related cardiovascular disease.

Cardiac Manifestations in Behçet's Syndrome / Bello, Federica; Bagni, Giacomo; Seyahi, Emire; Chiara, Emanuele; Olivotto, Iacopo; Saadoun, David; Emmi, Giacomo. - In: CURRENT RHEUMATOLOGY REPORTS. - ISSN 1534-6307. - STAMPA. - 27:(2025), pp. 31.1-31.16. [10.1007/s11926-025-01190-z]

Cardiac Manifestations in Behçet's Syndrome

Bello, Federica;Bagni, Giacomo;Chiara, Emanuele;Olivotto, Iacopo;Emmi, Giacomo
2025

Abstract

Purpose of the Review: Behçet’s Syndrome (BS) is a multisystemic vasculitis that can affect the heart, leading to pericarditis, myocarditis, intracardiac thrombosis, endomyocardial fibrosis, valvular dysfunction, and coronary artery disease. This review summarizes the clinical presentation, diagnostic challenges, and therapeutic strategies for cardiac involvement in BS. Recent Findings: Advanced imaging techniques have revealed subclinical cardiac involvement in BS. Myocardial dysfunction and fibrosis contribute to heart failure and arrhythmias, while intracardiac thrombi often coexist with pulmonary artery involvement. Coronary artery vasculitis and aneurysms may mimic atherosclerotic disease, complicating diagnosis. Biologic therapies, including TNF-α inhibitors, show promise in refractory cases. Summary: Early diagnosis and immunosuppressive therapy are crucial. A multidisciplinary approach is essential to managing cardiac complications and optimizing patient outcomes. Future research should refine screening protocols and explore targeted immunotherapies for BS-related cardiovascular disease.
2025
27
1
16
Goal 3: Good health and well-being
Bello, Federica; Bagni, Giacomo; Seyahi, Emire; Chiara, Emanuele; Olivotto, Iacopo; Saadoun, David; Emmi, Giacomo
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Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/1453175
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