Chapter 21 - Behçet’s Disease

Abstract

Behçet’s disease (BD) is a systemic inflammatory disorder whose clinical hallmark is recurrent oral and genital ulcers variably associated with skin and organ involvement. BD is currently considered systemic vasculitis. Vascular involvement is typically characterized by thrombotic events and occurs in about 30% of patients, while cardiac involvement has lower frequency, ranging from 1–6% to 16.5%. Valvulopathies, coronary arteritis with or without myocardial infarction, aneurysms of the coronary arteries, myocarditis, pericarditis, endomyocardial fibrosis, arrhythmia, intracardiac thrombus formation of the heart chambers or of the aortic sinus, and pulmonary artery aneurysm are all possible manifestations of BD. Timely detection of cardiac involvement is pivotal for appropriate management. Echocardiography is the most widely used diagnostic tool and may be complemented by cardiac MRI and/or CT angiography in selected cases. Treatment of cardiac BD is challenging and still largely empirical. High-dose glucocorticoids and immunosuppressive agents are recommended. In this chapter, we review the spectrum of cardiac manifestations in the course of BD. We also analyze the role of the different imaging techniques for an accurate diagnosis, treatment options, and cardiac implications of therapy.