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.