Granulomatous Pneumocystis carinii pneumonia in Wegener's granulomatosis

This study reports on a first case of granulomatous Pneumocystis carinii pn eumonia (PCP) in a human immunodeficiency virus-negative patient with antin eutrophil cytoplasmic antibody-positive Wegener's granulomatosis whilst rec eiving immunosuppressive treatment. The patient presented with diffuse alve olar haemorrhage, pauci-immune rapid progressive glomerulonephritis and leu kocytoclastic vasculitis of the skin. Granulomatous Pneumocystis carinii pn eumonia developed under immunosuppressive treatment with cyclophosphamide a nd prednisone. At the time Pneumocystis carinii pneumonia developed, there was a marked lymphopenia with a very low CD8+ cell count in the blood. Groc ott staining in bronchoalveolar lavage fluid revealed no Pneumocystis carin ii. The diagnosis was made via a video-assisted thoracoscopic lung biopsy w hich showed granulomas containing high numbers of Pneumocystis carinii cyst s.