Clinical and immunologic characteristics of Japanese patients with anti-glomerular basement membrane disease: case reports and literature review

Abstract Background Clinical studies of anti-glomerular basement membrane (GBM) disease were limited because the low incidence. We aimed to report characteristics, treatments, and outcomes patients with anti-GBM at a tertiary reference medical center in Japan review literature mortality disease. Case presentation 1 was 72-year-old Japanese man that referred worsening serum creatinine (from 1.1 27.3 mg/dL). Anti-GBM confirmed by renal biopsy, treatments oral prednisolone plasmapheresis initiated. Although his antibody decreased 476 18 units/mL) after patient died from lung abscess. 2 32-year-old presented fever macroscopic hematuria. At presentation, 4.2 mg/dL, 265 units/mL. Renal biopsy diagnosis disease, intensive methyl started, followed prednisolone. Living-donor kidney transplantation performed had remained undetectable for year diagnosis. In main text, clinicopathological characteristics 12 our institution summarized. Conclusions found 1-year survival rate 88% cohort, which comparable previous studies. Multicenter, nationwide are expected evaluate prognosis this rare entity.