Behcet's Disease: New Concepts in Cardiovascular Involvements and Future Direction for Treatment

Abstract

<jats:p>Behcet’s disease (BD) is the only systemic vasculitis involving both arteries and vein in any sizes. It frequently encounters in rheumatology clinics. It has some major morbidities and even fatal outcomes in some cases. The aim of this paper is to analyze the main concepts on pathophysiology and treatment options in BD, focusing on cardiovascular aspects, thrombosis, and potential future treatment.</jats:p>

Vascular Manifestations of Behçet’s Disease

“…Acute pericarditis, tamponade, and constrictive pericarditis have been reported. Myocarditis, cardiomyopathy causing diastolic and systolic dysfunction, valvular pathology coronary thromboses, coronary aneurysms, coronary rupture, predominantly right-sided intracardiac thrombus, aneurysm of the aorta, and its branches including the arch of aorta are other important cardiac complications of BD [112]. Several cardiac manifestations may occur in one patient [2].…”

“…Mortality is rather high (around 20%). Cardiac involvement in BD could be asymptomatic [112]. Cardiac involvement, when occurs, coexists with mucocutaneous manifestations.…”

“…Coronary aneurysms are more frequent than stenoses and can present as acute coronary syndrome and MI but sometimes are symptomatic [112]. In young adults with myocardial infarction, BD should be considered as a nonatherosclerotic cause of CAD.…”

“…It was reported that valvular prolapse including mitral valve prolapse can be related to vasculitis and tissue derangement [112].…”

A missed Behçet’s case presenting with spontaneous epidural hematoma

“…Deep vein thrombosis is the most frequent vascular involvement (2). Spinal vascular events related to Behçet' s disease are relatively uncommon (3).…”