Angio-behçet’s Disease with Concomitant Neurologic, Pulmonary and Cardiac Involvement: A Case Report

Concomitant neurological, cardiac, and pulmonary vascular involvement in Behçet’s disease is very rare and characterized by high mortality. Management is challenging because of the very high risk of bleeding. We report the case of a 25-year-old man, of Maghreb descent, who was admitted with acute motor deficit in all four limbs. A thoracic computed tomography angiogram (CTA) showed thrombosis of an aneurysm of the inferior lobar pulmonary arteries bilaterally, with an obstructive intraluminal thrombus of a segmental branch of the right superior lobe. Transthoracic echocardiography (TTE) revealed a mobile right atrial thrombus measuring 13 × 10 mm. The patient was treated with methylprednisolone (1 g), cyclophosphamide, colchicine, and low weight heparin followed by oral antivitamin K anticoagulation. The outcome was satisfactory with complete resolution of the atrial thrombus and the aneurysm, and mild neurological sequelae at six months.