GOODPASTURES-SYNDROME ASSOCIATED WITH PRIMARY BILIARY-CIRRHOSIS

A 73-year-old woman who had a history of primary biliary cirrhosis dev eloped rapidly progressive glomerulonephritis and pulmonary-renal synd rome. She was found to have antimitochondrial antibody (AMA) and myelo peroxidase (MPO) anti-neutrophil cytoplasmic antibody (ANCA). She also had weak anti-glomerular basement membrane (GBM) antibody. She was tr eated with methylprednisolone pulse therapy, but died of respiratory f ailure. On postmortem examination, both lungs showed diffuse hemorrhag e and the immunofluorescence study of the kidney revealed linear immun oglobulin G (IgG) deposition along the glomerular basement membrane.