Last update:
04-Apr-2013

The case is presented of superior vena cava syndrome (SVCS) and intracardiac thrombosis as the initial manifestations of Adamantiades-Behçet's disease. A 32-year-old man was admitted to the department of internal medicine with progressive dyspnea, dizziness, headache and edema of the face and the neck. Computed tomography (CT) initially, and digital subtractive angiography showed extensive thrombosis in the superior vena cava and right subclavian vein. Adamantiades-Behçet's disease was diagnosed on the basis of clinical criteria, namely recurrent aphthous stomatitis, genital ulceration and episcleritis, with a positive pathergy test and identification of the HLA B51 allele. Conservative treatment was initiated with oral azathioprine, steroids and anticoagulant agents. Two months later, transesophageal echocardiogram revealed a sizeable pedunculated thrombus oscillating in the right atrial, which was removed surgically. The patient was discharged and was prescribed oral azathioprine, aspirin, acenocoumarol and corticosteroids with tapering dosage. Ten-year follow-up has been maintained, including echocardiography, CT angiography and magnetic resonance venography. Complete resolution of the thrombi was obtained in 2 years and no recurrence of the disease has been observed. Venous thrombosis represents the most frequent lesion of vasculo-Behçet's disease, but SVCS and particularly intracardiac thrombosis are rare manifestations which usually appear late in the course of the disease.

Key words: Adamantiades-Behçet's disease, Intracardiac thrombosis, Superior vena cava syndrome.