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Anti-glomerular basement membrane antibody disease in Japan: part of the nationwide rapidly progressive glomerulonephritis survey in Japan

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Abstract

Anti-glomerular basement membrane (anti-GBM) antibody disease is a rare, but well characterized cause of glomerulonephritis. It is defined by the presence of autoantibodies directed at specific antigenic targets within the glomerular basement membrane. This pattern of rapidly progressive glomerulonephritis and alveolar hemorrhage is often referred to as Goodpasture’s syndrome. The prognosis for patients with anti-GBM antibody disease is poor. In Japan, to improve the prognosis of patients with rapidly progressive glomerulonephritis (RPGN), we conducted a nationwide survey of patients with RPGN and investigated the initial symptoms, laboratory findings including renal biopsy findings, treatment methods, and outcomes. Among patients with RPGN, patients with anti-GBM antibody disease were rare: 6.6% (47/715). Alveolar hemorrhage (Goodpasture’s syndrome) was observed in 23.4% of patients with anti-GBM antibody disease. Most patients with anti-GBM antibody disease had renal failure at the time of diagnosis. The mean serum creatinine level of patients with renal-limited anti-GBM antibody disease was 7.07 ± 4.21 mg/dl and that of patients with Goodpasture’s syndrome was 7.99 ± 4.31 mg/dl. The mean level of crescent formation was 78.99 ± 23.54% in patients with anti-GBM antibody disease, and a cellular crescent form was observed in 63.2% of those patients. The prognosis for patients with anti-GBM antibody disease is poor; the renal survival rate at 6 months after onset was 20.9%, and the mortality at 6 months after onset was 23.3%. To improve the prognosis for anti-GBM antibody disease, it may be necessary to detect this disease in the early stages and to treat it without delay.

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Acknowledgements

We acknowledge the people who participated in this Japanese nationwide survey and the members of the RPGN Clinical Guidelines Committee of Japan. The Japanese nationwide RPGN survey was supported by a grant-in-aid from the Research Fund for the Special Study Group on Progressive Glomerular Disease, the Ministry of Health, Labour and Welfare, Japan. The abstract was presented in Japanese at the 36th East-Japan annual meeting of the Japanese Society of Nephrology on 3 November 2006.

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Correspondence to Kouichi Hirayama.

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Presented at the 36th Eastern Regional Meeting of the Japanese Society of Nephrology.

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Hirayama, K., Yamagata, K., Kobayashi, M. et al. Anti-glomerular basement membrane antibody disease in Japan: part of the nationwide rapidly progressive glomerulonephritis survey in Japan. Clin Exp Nephrol 12, 339–347 (2008). https://doi.org/10.1007/s10157-008-0051-8

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