Anti-glomerular basement membrane antibody disease : a case report and a review of Japanese patients with and without alveolar hemorrhage

A 73-year-old man was admitted to our hospital because he had developed loss of appetite, abnormal behaviors, and consciousness disturbances that had begun in late February 1998. On admission, a renal biopsy was performed because of progressive deterioration of renal function, as evidenced by a serum urea nitrogen (UN) level of 109 mg/dl and a serum creatinine level of 16.3 mg/dl. Light microscopic examination showed severe cellular crescent formation with fibrin deposition in glomeruli and markedly degenerated Bowman's capsule. Immunofluorescence examination revealed linear deposits of IgG along glomerular basement membranes (GBM), and granular deposits of C3 on the GBM, as well as deposits of fibrinogen in Bowman's capsule. The patient was diagnosed as having anti-GBM antibody disease, based on negative results for myeloperoxidase (MPO)/proteinase-3 (PR-3)-anti-neutrophil cytoplasmic antibody (ANCA), high serum anti-GBM antibody titers, and the absence of pulmonary hemorrhage. He was treated with both combination therapy (cyclophosphamide and prednisolone) and plasmapheresis. In spite of the disappearance of anti-GBM antibody, his renal function did not improve, and he has been treated with regular hemodialysis since March 1998. We reviewed 49 cases of anti-GBM antibody disease in patients with alveolar hemorrhage (group A; Goodpasture's syndrome) and 39 cases in patients without it (group B) reported in Japan from 1975 to 1999, examining the differences between these groups, and we clarified the characteristics of these rare diseases in Japan. There was no difference in age, sex, and ANCA positivity between the two groups. The mortality rate was higher in group A (56.2%) than in group B (18.4%). About half of the patients underwent plasmapheresis, but it did not reduce the mortality rate or improve the renal prognosis.