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