Intracardiac thrombosis and vascular involvement in Behcet's syndrome: two sides of the same coin?

Abstract

Abstract Background Behçet’s syndrome (BS) is a complex and relapsing vasculitis that affects vessels of all kinds and sizes. Intracardiac thrombosis (ICT) is a rare but serious complication of the disease, that usually presents with vascular lesions. However, its pathogenesis remains unclear and the link between these manifestations is yet not fully clarified. Objectives Our work aims to describe the clinical characteristics, imaging features, therapeutic management, and outcomes of BS patients with ICT. And secondly, to shed some light on the relation between ICT and the vascular involvement of the disease. Methods We retrospectively conducted a descriptive and monocentric study including 531 BS patients admitted to our department between 2010 and 2022. Results Twelve cases of ICT were enrolled (2%). Patients were mostly males (91.6%), with a mean age of 29 ± 6 years (20 – 41). Past history of lower extremity deep vein thrombosis (DVT) related to BS was frequently reported (41%). ICT was the revealing manifestation of the disease in 50% of the cases. In the other half, it occurred after a median of 4 years following BS onset. Symptoms were dyspnea (50%), chest pain (50%), dry cough (40%), and hemoptysis (30%). Signs of right (33%) and global (17%) heart failure were found. Concomitant noncardiovascular manifestations were mainly mucocutaneous (80%). Inflammatory parameters were always raised with a median ESR of 48 mm/h and CRP of 52 mg/l. Hemostasis parameters were within the normal ranges for all patients. High levels of BNP and Troponin were detected in respectively 50% and 16% of the cases. On echocardiography, ICT was located in the right heart (100%), with an extension to the inferior vena cava (50%). Multiple thrombi were visualized in three cases. The associated cardiac lesions were pericardial effusion (50%), tricuspid (50%) and mitral insufficiency (10%), endomyocardial fibrosis (10%), myocarditis (10%), and ischemic coronaropathy (8%). Pulmonary hypertension (75%) and right dilatation (58%) were frequent, with persevered systolic function (100%). On Chest CTA, pulmonary artery thrombosis (70%) and aneurysms (50%) were observed. Peripheral DVT was also common (41%). All patients were treated with colchicine, glucocorticoids, and immunosuppressants: cyclophosphamide (n=11), and TNF-alpha inhibitor (n=1). Curative anticoagulation was prescribed in half of the cases. Outcomes were: complete remission (n=9), partial remission (n=2), and relapse (n=1). No death was recorded. Conclusion ICT in BS is a life-threatening event that occurs in the first years of the disease onset, among young men with vascular lesions, especially of the pulmonary arteries and vena cava. We believe that this strong association is highly suggestive that, unlike other thrombotic diseases, ICT in BS is a direct consequence of the underlying vasculitis. Hence, prompt and adequate management with immunosuppressive therapy is usually associated with good outcomes.Clinical baseline characteristicsCardiac and vascular manifestations