Behçet’s disease presenting with intracardiac thrombus and pulmonary thromboembolism in a young Ethiopian male: a case report

Abstract

INTRODUCTION: Behçet's disease (BD) is a rare, chronic, relapsing multisystem vasculitis with diverse manifestations, including mucocutaneous, ocular, neurological, and vascular involvement. Intracardiac thrombus is a rare manifestation of BD and can result in severe complications such as pulmonary thromboembolism (PTE). CASE PRESENTATION: A 26-year-old Ethiopian male with a history of recurrent oral and genital ulcers and a prior episode of deep venous thrombosis (DVT) presented with hemoptysis and exertional dyspnea of two days. Physical examination revealed tachycardia and tachypnea, multiple healed ulcerations on the scrotum and penile shaft. He had an antinuclear antibody (ANA) titer of 1:320. A right ventricular (RV) apical thrombus measuring 2.8 × 2.1 cm on echocardiography and bilateral segmental and sub-segmental pulmonary emboli on computed tomography (CT) pulmonary angiography. The diagnosis of BD was made based on the International Criteria for BD, with a total score of 5. The patient was treated with anticoagulation and immunosuppressive therapy, including heparin, warfarin, prednisolone, and cyclophosphamide, resulting in clinical improvement and resolution of symptoms. CONCLUSION: This case highlights a rare but serious cardiovascular presentation of BD in a region where it is seldom reported. It underscores the importance of clinical vigilance for BD in young patients with unexplained thrombotic events. Early diagnosis and appropriate immunosuppressive and anticoagulant therapy are essential for improving outcomes.