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: Nagasu Hajime, Abe Michiko, Kuwabara Atsunori, Kawai Tatsuo, Nishi Yuko, Okuda Naoki, Sakaguchi Katsuhiko
• Journal: Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy (Ther Apher Dial) Vol. 13 Issue 4 Pg. 373-7 (Aug 2009) ISSN: 1744-9987 [Electronic] Australia
• PMID: 19695077 (Publication Type: Case Reports, Journal Article)

Chemical References

• Autoantibodies
• Immunoglobulin G
• Immunosuppressive Agents
• antiglomerular basement membrane antibody
• Cyclophosphamide
• Methylprednisolone

Topics

• Adult
• Anti-Glomerular Basement Membrane Disease (diagnosis, immunology, therapy)
• Autoantibodies (immunology)
• Cyclophosphamide (therapeutic use)
• Humans
• Immunoglobulin G (immunology)
• Immunosuppressive Agents (therapeutic use)
• Male
• Methylprednisolone (therapeutic use)
• Plasmapheresis (methods)