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Seven patients presented a specific renal lesion of rapidly progressive glomerulonephritis with myeloperoxidase anti-neutrophil cytoplasmic antibody. Rapidly progressive glomerulonephritis with myeloperoxidase anti-neutrophil cytoplasmic antibody can be associated with pulmonary hemorrhage and/or pulmonary interstitial fibrosis. The patients included three men and four women with a mean age of 62.4 years. All courses of renal lesions revealed rapidly progressive glomerulonephritis and the serum creatinine levels were 7.1 ± 3.9 mg/dl and the myeloperoxidase anti-neutrophil cytoplasmic antibody was 473 ± 471 EU before treatments. Steroid therapy was administered to all patients, immunosupressive agent to four and hemodialysis in six cases. Four patients experienced a pulmonary hemorrhage and died, but all of three patients with pulmonary interstitial fibrosis survived.