A FATAL CASE OF BEHCETS-DISEASE ASSOCIATED WITH MULTIPLE CARDIOVASCULAR LESIONS

Behcet's disease is recognised as a chronic multisystem disorder with vasculitis as its underlying pathological process. Cardiac involvement is rare and often associated with poor prognosis. A large right atria l thrombus, pulmonary aneurysms and aortic pseudoaneurysm that develop ed 17 years after surgery for bilateral renal artery stenosis is prese nted in a 26-year-old Behcet's disease patient. He was admitted to the hospital with fever of unknown origin associated with chest pain, dys pnea, cough, haemoptysis and pulmonary opacity in chest X-ray. Initial pulmonary CT demonstrated small subpleural infiltrates bilaterally, o ne of which was round and suspected as being metastatic. Examination o f open lung biopsy demonstrated haemorrhagic infarct surrounded by som e occluded pulmonary arteries. Subsequent CT showed pulmonary aneurysm s compatible with Behcet's disease. Echocardiography demonstrated a la rge pedunculated mass in the right atrium. Injection of urographin sho wed a right atrial mass and a large right pulmonary artery aneurysm. T he atrial mass was completely excised during open heart surgery and wa s identified as being an organising thrombus. Eight weeks later while taking prednisone, he was readmitted because of an infected mid sterna l wound. CT showed slight separation of the sternum, retrosternal flui d, pulmonary arteries aneurysm and ascending aorta aneurysm. The next day, the patient died from massive bleeding from his ruptured ascendin g aortic pseudoaneurysm. Bizarre presentation of arterial and venous t hromboses or arterial aneurysm formation, particularly in young patien ts, should suggest Behcet's disease. (C) 1997 Elsevier Science Ireland Ltd.