Background Background: Behçet's disease (BD) is a chronic, multisystemic disorder characterized by recurrent oral aphtous ulcers, genital ulcers and ocular inflammation. Vasculitis and thrombotic events are the most important causes of mortality. Vena cava thrombosis, pulmonary artery aneurysms, Budd-Chiari syndrome, peripheral artery aneurysms, dural sinus thrombosis and abdominal aorta aneurysms are the other less common vascular manifestations of BD. Cardiac involvement in BD is a rare and life-threatening complication.
Objectives Objective: The aim of this study was to assess the clinical characteristics and outcome of patients with BD who have intracardiac thrombus.
Methods The hospital files of BD patients followed by rheumatology clinics of four medical centers (Ankara University, Ege University, Hacettepe University and Eskişehir Osmangazi University Hospitals) were retrospectively evaluated. Data included patients' demographic data, clinical features, laboratory findings and outcome. All patients fulfilled three or more of the International Study Group Criteria for BD.
Results Results: Twenty-two patients with intracardiac thrombus were evaluated. Mean age of patients with intracardiac thrombosis was 29.1 yrs (22-44) and there was a male predominance with a ratio of 20:2. Cardiac involvement was the first clinical manifestation of BD in 9 of the 22 patients. Initial symptoms of the patients were fever (n=18, 81%), dyspnea (n=9 40%) chest pain (n=9, 40%) and hemoptysis (n=7, 31.8%). Sixteen patients (72%) had pulmonary thromboembolism (Figure 1) and extra-cardiopulmonary vascular involvements included deep vein, inferior vena cava, hepatic vein, renal artery thrombosis (n=11, 50%). Intra-cardiac thrombus were found only in the right cavities in 17 patients (77%), only in the left cavities in 2 patients (9%), and in both left and right cavities of the heart in 3 patients (13.6%). Once the cardiac lesion was diagnosed as a complication of BD, high dose (1mg/kg/d) prednisone (n=22, 100%) plus cyclophosphamide (n=18, 81%) or azathioprine (n=3, 13.6%) and warfarin (n=8, 36.3% (after the elimination of pulmonary aneurism) therapy for anticoagulation was initiated. Four patients (18%) had high dose prednisone plus cyclophosphamide plus interferon-α (IFN-α) combination treatment and two patients (n=2, 9%) had high dose prednisone plus cyclophosphamide or plus azathioprine combination treatment.Only two patients underwent surgery because of intra-cardiac thrombus. After treatment, the intra-cardiac thrombus disappeared in 13 cases during the nine month of therapy and the size of the thrombus decreased in 7 cases.
Conclusions Conclusion: Intracardiac thrombus in BD is more common in young men. The right side of the heart is usually involved and cardiac involvement is often accompanied by pulmonary artery occlusion possibly due to pulmonary arteritis. Early and aggressive immunosuppresive and/or anticoagulation therapy are life-saving.
Disclosure of Interest None declared
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