Rare manifestation of Behcet's syndrome: insight from multimodality cardiovascular imaging

Abstract

SummaryA young Jordanian man was hospitalised with fever of 2 months duration. Preadmission diagnostic assessment including CT thorax and abdomen were inconclusive. Right-sided pleuritic chest pain was present on admission. Pulmonary embolism was suspected on a ventilation/perfusion lung scan. Lower-extremity deep venous thrombosis was, however, absent. Echocardiogram to exclude a cardiac source of pulmonary embolism showed a right ventricle mass which was also present on retrospective review of prehospitalisation CT thorax. Tissue characterisation of this mass on cardiac MRI was not helpful. Empiric anticoagulation was started without reduction in size at 3 weeks. Due to ongoing diagnostic uncertainty, surgical resection was performed. Histopathology confirmed this mass to be a thrombus. With unabated fever, right ventricular thrombosis with pulmonary embolism and cutaneous and scrotal ulceration which evolved later in the hospital course, a diagnosis of Bechet's syndrome was considered and subsequently confirmed. Response to immunosuppressive therapy was prompt. BACKGROUND