A 73-year-old woman who had a history of primary biliary cirrhosis developed rapidly progressive glomerulonephritis and pulmonary-renal syndrome. She was found to have anti-mitochondrial antibody (AMA) and myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibody (ANCA). She also had weak anti-glomerular basement membrane (GBM) antibody. She was treated with methylprednisolone pulse therapy, but died of respiratory failure. On postmortem examination, both lungs showed diffuse hemorrhage and the immunofluorescence study of the kidney revealed linear immunoglobulin G (IgG) deposition along the glomerular basement membrane.
(Internal Medicine 37: 611-613, 1998)