Cardiovascular Involvement in Behçet Disease: Clinical Implications

Abstract

Behçet disease (BD) is a multisystem disorder, with vasculitis as its underlying pathological process, in contrast to the classic triad of recurrent oral and genital ulcerations, with uveitis. Vascular involvement in BD includes venous thrombosis, arterial occlusion, and pulmonary artery and aortic aneurysm formation. Cardiac involvement is rare and often obscure. It includes intracardiac thrombi formation, and is associated with a poor prognosis. Our objectives are to describe two cases with BD, complicated with vascular and cardiac involvement, and to raise awareness of these rare complications, the needed routine surveillance, and thus to prevent inappropriate interventions, serious outcomes, and mortality. We present two male patients from the Mediterranean Basin with BD. The first was diagnosed early as a BD patient. The second was diagnosed at the time of cardiovascular (CV) involvement. We recommend that patients who are diagnosed, or even suspected of suffering from BD, especially in endemic areas along the Silk Route pathway, should be followed up routinely for CV involvement, even if rare, obscure, or with a bizarre presentation.

Keywords

Behçet disease - cardiovascular involvement - vasculitis - pulmonary embolism - intracardiac thrombus

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