An autopsy case of refractory vasculo-Behçet's disease

Abstract

Pulmonary vascular involvement in Behçet's disease is a rare complication with a poor prognosis. We present an autopsy case of vasculo-Behçet's disease complicated by pulmonary hemorrhage, possibly caused by rupture of pulmonary artery aneurysms. The patient was treated with a combination of high-dose steroids and pulse cyclophosphamide, but he died from massive hemoptysis. This case highlights the need for potent new therapies for patients with vasculo-Behçet's disease refractory to conventional immunosuppressive therapy, such as a combination of steroids and cyclophosphamide.

Keywords: Immunosuppressive therapy; Pulmonary artery aneurysm; Vasculitis; Vasculo-Behçet disease.