title: A case report of efficiency of double filtration plasmapheresis in treatment of Goodpasture's syndrome.
abstract: Goodpasture's syndrome is characterized by pulmonary hemorrhage, rapid progressive glomerulonephritis and the presence of anti-glomerular basement membrane (anti-GBM) antibodies. Here, we report a case of Goodpasture's syndrome that we treated with double filtration plasmapheresis (DFPP) combined with immunosuppression therapy. The patient was a 32-year-old man with the main complaints of low-grade fever, general fatigue and dyspnea. The clinical diagnosis was renal-pulmonary syndrome based on pulmonary hemorrhage on chest X-ray, rapid progressive renal insufficiency, and elevated C-reactive protein (CRP). Goodpasture's syndrome was diagnosed because the patient was negative for MPO-ANCA and PR3-ANCA, and positive for anti-GBM antibodies. Renal biopsy showed crescentic glomerulonephritis. Hemodialysis, immunosuppression therapy (methylprednisolone and cyclophosphamide) and DFPP were performed. Anti-GBM antibodies were followed pre- and post-DFPP, and removal efficiency, cost performance and complications were evaluated. The antibody levels were 121 and 84 EU/mL before and after the first DFPP procedure, respectively, giving a removal efficiency of 24%. Subsequently, the removal efficiencies were 52%, 55% and 60% after the second, third and fourth DFPP procedures. For comparison, the immunoglobulin G (IgG) removal efficiencies were 53%, 57%, 60% and 55% after the four respective DFPP procedures; therefore, the removal efficiencies were similar for anti-GBM antibodies and IgG in all except the first DFPP procedure. The serum anti-GBM antibody and IgG concentrations decreased from pre- to post-DFPP, indicating that DFPP may be an effective therapeutic approach in Goodpasture's syndrome.
authors.affiliation: Department of Nephrology, Sumitomo Hospital, Osaka, Japan. zajiten@med.kawasaki-m.ac.jp
authors.forename: Nagasu
authors.initials: N
authors.lastname: Hajime
authors.forename: Abe
authors.initials: A
authors.lastname: Michiko
authors.forename: Kuwabara
authors.initials: K
authors.lastname: Atsunori
authors.forename: Kawai
authors.initials: K
authors.lastname: Tatsuo
authors.forename: Nishi
authors.initials: N
authors.lastname: Yuko
authors.forename: Okuda
authors.initials: O
authors.lastname: Naoki
authors.forename: Sakaguchi
authors.initials: S
authors.lastname: Katsuhiko
mesh_terms: D000328:Adult; D019867:Anti-Glomerular Basement Membrane Disease / Q000175:diagnosis / Q000276:immunology / Q000628:therapy*; D001323:Autoantibodies / Q000276:immunology*; D003520:Cyclophosphamide / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D010956:Plasmapheresis / Q000379:methods*
chemical_list: D001323:Autoantibodies; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; C051951:antiglomerular basement membrane antibody; D003520:Cyclophosphamide; D008775:Methylprednisolone