Crescentic Glomerulonephritis with Positive Antineutrophil Cytoplasmic Autoantibody Specific for Myeloperoxidase Associated with Autoimmune Hemolytic Anemia and Thrombocytopenic Purpura

A 41-year-old woman was admitted to the hospital with severe uremia, hemolytic anemia, and thrombocytopenic purpura. Emergency heniodialysis with plasmapheresis was started in view of consideration of hemolytic uremic syndrome (HUS), which resulted in improvement of renal function and platelet count. Positive antineutrophil cytoplasmic autoantibody specific for myeloperoxidase (MPO-ANCA) suggested crescentic glomerulonephritis, which was pathologically evidenced by renal biopsy. The diagnosis of MPO-ANCA associated crescentic glomerulonephritis with autoimmune hemolytic anemia (AIHA) and thrombocytopenic purpura were comfirmed. Three courses of steroid pulse therapy with heparin were successfully performed, followed by oral prednisolone and warfarin. Such a case has not been previously reported to our knowledge. (Internal Medicine 39: 650-654, 2000)