Intracardiac thrombus in Behçet’s disease

Abstract

Behçet’s disease (BD) is a multisystem inflammatory disorder. Intracardiac thrombus (ICT) formation is an uncommon but important complication of BD. Of the cases of Behçet’s disease, we selected those with ICT. All patients fulfilled the diagnostic criteria of the International Study Group of Behçet’s disease. The ICT in each case was confirmed by ultrasonography, computed tomography and MRI. Clinical features and laboratory parameters were determined. Among our 518 patients with BD, 8 were diagnosed as having intracardiac thrombus (ICT). All were male; the mean age at the time of the ICT diagnosis was 30.8 years. The main presenting symptoms were hemoptysis, chest pain, and dyspnea. It was associated with pulmonary artery aneurysm and vena cava thrombosis in 3 cases each, pulmonary embolism, and lower limbs deep venous thrombosis in 1 case each. The coexistence of other cardiac complications was as follows: pericarditis in 2 cases, myocarditis, endomyocardial fibrosis, and coronary arteritis with consequent myocardial infarction in one case each. In all cases, echocardiography was sufficient to reach the diagnosis. Chest computed tomography performed in all cases led to the diagnosis of associated pulmonary vasculo-Behçet lesions in 4 cases. All patients received colchicine, anticoagulation, and corticosteroids. Seven patients were on immunosuppressant agents (2 patients received azathioprine and 5 cyclophosphamide). Clinical remission with ICT resolution was observed in 5 cases. Combined immunosuppressive therapy with prednisone and cyclophosphamide might be needed to treat ICT due to BD.

Vascular Manifestations of Behçet’s Disease

“…EULAR does not recommend anticoagulants in the treatment of thrombosis [121]. Unfortunately, as of now, there are no controlled studies or evidence of benefit from experience with anticoagulants, antiplatelet, or antifibrinolytics in vascular BD [129].…”

“…Corticosteroids plus immunosuppressants reduce the thrombus formation and improve aortic regurgitation and heart failure in cardiac BD, whereas surgery alone does not lead to complete resolution of thrombus [129].…”

“…ICT has risk of recurrence [129]. Combined immunosuppressive therapy with steroids (prednisolone) and cyclophosphamide is needed to treat ICT and prevent recurrences.…”

“…It is important to know that thrombus disappears after the initiation of immunosuppressive therapy [129]. Medical treatment with immunosuppressants may be the first choice for patients with BD who have ICT [111].…”

“…To avoid a progression to thrombus or cardiac dysfunction in recurrent cases, the early identification of cardiac involvement of BD using echocardiography and/or cardiac magnetic resonance imaging is of great importance. Combined immunosuppressive therapy with prednisolone and cyclophosphamide are usually needed to treat recurrent thrombosis due to BD [111,129].…”