Abstract
We present three patients with Behçet's disease associated with intracardiac thrombus and pulmonary vascular involvement. One of these patients had also Budd-Chiari syndrome. All patients were treated with corticosteroid plus monthly intravenous cyclophosphamide as first line treatment and with no recurrences. Immunosuppressive therapy was successful in the treatment of intracardiac thrombus and also in the regression of pulmonary vascular thromboses in these patients. Intracardiac thrombus in Behçet's disease is rarely seen. Behçet's disease should be remembered in the differential diagnosis of the patients with intracardiac mass, especially in patients from the Mediterranean and Middle East populations.
Highlights
Behcet’s disease (BD) is a multisystemic, chronic, inflammatory disorder of unknown etiology
A 25-year-old Turkish man who had been followed for one year with a diagnosis of Budd-Chiari syndrome was admitted to the rheumatology clinic with fever up to 40∘C and abdominal pain
Intracardiac thrombosis is an uncommon complication of BD
Summary
Behcet’s disease (BD) is a multisystemic, chronic, inflammatory disorder of unknown etiology. Behcet’s disease is recognized as a systemic vasculitis involving both arteries and veins of any size. Most vascular events consist of recurrent superficial or deep vein thrombosis [2,3,4]. Arterial thrombosis is less frequent [3]. The cardiac findings were found in 1%–6% of BD cases in previous clinical series [5] and in 16.5% of cases in the Japanese autopsy registry [6]. Cardiac manifestations of BD include endocarditis, myocarditis, pericarditis, endomyocardial fibrosis, coronary arteritis with or without myocardial infarction, aneurysms of the coronary arteries, valve dysfunction, conduction system disturbances, and intracardiac thrombosis (ICT) [5, 7,8,9]. We present the characteristic clinical findings in three cases with ICT related to BD and review the approach to treatment of ICT in BD patients
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