Schwere Hämoptoe bei pulmonaler Vaskulitis

Abstract

A 27-year-old man, in good health but a moderate smoker, suddenly had two episodes of haemoptysis. Routine clinical examination was unremarkable. Erythrocyte sedimentation rate was increased to 34 mm/h. The chest radiography showed ill-defined, contrast-poor infiltrations bilaterally, as well as left hilar enlargement. Lung scintigraphy and pulmonary arteriogram suggested pulmonary embolism, possible from a "pelvic vein spur", i.e. an intimal proliferation due to crossing of the common iliac artery over the pelvic vein. He was placed on oral anticoagulants. Three months later he had another severe haemoptysis, providing the indication for an exploratory thoracotomy. This revealed the left pulmonary artery wall to have inflammatory changes with aneurysmal dilatation. The aneurysm was plicated. Histological examination demonstrated chronic vasculitis as seen in Behçet's syndrome, a diagnosis confirmed by the findings of ulcers of the oral mucosa and the presence of HLA B5 allo-antigens. Immunosuppressive treatment was given with prednisone (1 mg/kg), azathioprine (2.5 mg/kg) and ciclosporin (5 mg/kg). Over the next 12 months there has been only one further haemoptysis.