Cardiac involvement in Behçet disease presenting as non-bacterial thrombotic endocarditis: A case report

Abstract Behcet disease (BD) is a form of widespread vasculitis that involves both arteries and veins. Cardiac involvement in BD is exceedingly rare and can present as a form of non-bacterial- thrombotic-endocarditis (NBTE). A 54-year-old man with Behcet disease was admitted to our hospital after presenting with abdominal pain and fever. He had been recently discharged from a hospital in another country with the diagnosis of infective endocarditis of the mitral valve and mycotic embolization to the superior mesenteric artery. At presentation, oral and genital ulcers were present, raising the suspicion of a flare of BD. Transesophageal echocardiography showed a small vegetation on the anterior leaflet of the mitral valve. Blood cultures results were negative. Computed tomography of the abdomen showed extensive inferior vena cava thrombosis. The aneurysm and thrombotic phenomena were interpreted as related to BD: the vegetation on the mitral valve was diagnosed as NBTE of which BD is a recognized cause. With corticosteroid and anticoagulant therapy, the patient's symptoms steadily improved. NBTE is a rare manifestation of BD; differential diagnosis with infective endocarditis can be challenging and should be focused on identifying predisposing conditions. The mainstay of treatment in NBTE is medical therapy with anticoagulation and treatment of the underlying disease. Learning objective Cardiac involvement in the setting of Behcet Disease (BD) is rare and several different manifestations are described (intracardiac thrombus, non-infective endocarditis, myocarditis, pericarditis, endomyocardial fibrosis, coronary arteritis). Non-bacterial thrombotic endocarditis (NBTE) is also a rare finding which can happen in the setting of BD. Its prevalence is unknown and evidence is derived only from scarce case reports. The role of BD as a potential cause of NBTE must be acknowledged to avoid potential misdiagnosis.