We report a 79-year-old woman with ANCA-related vasculitis. Rapid lung and renal damage were observed. Myeloperoxidase ANCA (MPO-ANCA) was as high as 640 times normal, and the patient died. ANCA-related disease should be considered when severe lung/renal damage with sudden onset is observed. This was a case in which the autopsy results confirmed our diagnosis of ANCA-related vasculitis.
