title: [Case of goodpasture syndrome associated with minimal change nephrotic syndrome (MCNS) in a patient with rheumatoid arthritis (RA)].
abstract: In early June 2004, a 50-year-old female was admitted to the hospital for slight fever, general fatigue, hemoptysis, dyspnea, and renal dysfunction (serum creatinine[Cr] : 6.05 mg/dL). She had been treated with prednisolone (PSL : 10-20 mg/day) for RA. She was diagnosed with Goodpasture syndrome based on a high titer of anti-glomerular basement membrane antibody (87 EU), and pulmonary hemorrhage. The renal and pulmonary impairments were markedly improved by the pulse therapy, plasma exchange and temporary hemodialysis. However, the Cr level remained at 2.0 mg/dL, indicating nephrotic syndrome. Light microscopy with Periodic acid-Shiff(PAS) staining demonstrated global sclerosis in three of ten glomeruli. Five glomeruli showed the formation of cellular, and fibrocellular crescents, and the formation of fibrous crescents. Tubular damage and interstitial fibrosis were severe. Immunofluorescence microscopy disclosed major depositions of IgG in a linear pattern along the glomerular basement membrane(GBM). Electron microscopy revealed foot process effacement (>50%)and no electron-dense deposits. Therefore, we diagnosed Goodpasture syndrome associated with minimal change nephrotic syndrome (MCNS). Some reports have dealt with the association of RA and Goodpasture syndrome with D-penicillamine, and of RA and antineutrophil cytoplasmic antibodies (ANCA)-related vasculitis with pulmonary hemorrhage, but none has dealt with cases complicated with RA and Goodpasture syndrome associated with MCNS. Accordingly, whether or not there is a causal relationship between RA and Goodpasture syndrome remains obscure, but since the number of reported cases is small, experience with more cases is necessary to clarify this matter.
authors.affiliation: Division of Nephrology and Kidney Center, Kobe University School of Medicine, Hyogo, Japan.
authors.forename: Yoko
authors.initials: Y
authors.lastname: Takeda
authors.forename: Aya
authors.initials: A
authors.lastname: Abe
authors.forename: Takeshi
authors.initials: T
authors.lastname: Toki
authors.forename: Hirotaka
authors.initials: H
authors.lastname: Komaba
authors.forename: Takaya
authors.initials: T
authors.lastname: Abe
authors.forename: Michio
authors.initials: M
authors.lastname: Umezu
authors.forename: Kensuke
authors.initials: K
authors.lastname: Joh
authors.forename: Masafumi
authors.initials: M
authors.lastname: Fukagawa
mesh_terms: D019867:Anti-Glomerular Basement Membrane Disease / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D001172:Arthritis, Rheumatoid / Q000150:complications* / Q000188:drug therapy; D005260:Female; D006801:Humans; D007668:Kidney / Q000473:pathology; D008775:Methylprednisolone / Q000008:administration & dosage; D008875:Middle Aged; D009402:Nephrosis, Lipoid / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D010951:Plasma Exchange; D011239:Prednisolone / Q000008:administration & dosage; D020551:Pulse Therapy, Drug; D006435:Renal Dialysis; D016896:Treatment Outcome
chemical_list: D011239:Prednisolone; D008775:Methylprednisolone