MPO-ANCA-positive anti-glomerular basement membrane antibody disease successfully treated by plasma exchange and immunosuppressive therapy.

Publisher: Informa Healthcare Country of Publication: England NLM ID: 8701128 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1525-6049 (Electronic) Linking ISSN: 0886022X NLM ISO Abbreviation: Ren Fail Subsets: MEDLINE

Publication: London : Informa Healthcare
Original Publication: New York, N.Y. : M. Dekker, c1987-

Antibodies, Antineutrophil Cytoplasmic/*immunology ; Autoantibodies/*immunology ; Glomerulonephritis/*immunology ; Hemorrhage/*immunology ; Immunosuppression Therapy/*methods ; Lung Diseases/*immunology ; Peroxidase/*immunology ; Plasma Exchange/*methods ; Plasmapheresis/*methods; Antibodies, Anti-Idiotypic/immunology ; Biopsy ; Bronchoalveolar Lavage Fluid/cytology ; Bronchoscopy ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Glomerulonephritis/diagnosis ; Glomerulonephritis/therapy ; Hemorrhage/diagnosis ; Hemorrhage/therapy ; Humans ; Immunoglobulin G/immunology ; Immunosuppressive Agents/therapeutic use ; Kidney Glomerulus/immunology ; Kidney Glomerulus/pathology ; Lung Diseases/diagnosis ; Lung Diseases/therapy ; Middle Aged ; Tomography, X-Ray Computed

Anti-glomerular basement membrane (GBM) antibody disease is clinically manifested as rapidly progressive glomerulonephritis (RPGN) with crescentic changes. The renal prognosis is poor. We report here the case of a 61-year-old woman with myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-positive anti-GBM antibody disease. This patient was referred to our hospital because of RPGN. Anti-GBM antibody was positive with a titer of 38 EU. The MPO-ANCA titer was 65 EU. Chest imaging examination revealed pulmonary multiple nodules. ANCA-associated vasculitis was suspected. Renal pathology revealed cellular crescents in 13 out of 17 glomeruli. Immunofluorescence with anti-IgG antibody, anti-C3 antibody, and anti-fibrin antibody showed linear staining along the glomerular capillary walls. Based on these findings, the patient was diagnosed with anti-GBM antibody disease. Hemodialysis was started because of uremic syndrome with elevated serum creatinine (6.84 mg/dL). In addition, treatment with plasma exchange using 3.6 L (90 mL/kg) of fresh frozen plasma combined with an oral dose of 40 mg of prednisolone was initiated. Within 3 weeks, both types of autoantibodies became undetectable. Subsequently, this patient achieved dialysis independence and remission of glomerulonephritis. No adverse effects were observed. In patients with MPO-ANCA-positive anti-GBM antibody disease, intensive therapy predominantly with plasma exchange might be operative, even though renal function is less likely to recover.

0 (Antibodies, Anti-Idiotypic)
0 (Antibodies, Antineutrophil Cytoplasmic)
0 (Autoantibodies)
0 (Immunoglobulin G)
0 (Immunosuppressive Agents)
0 (anti-IgG)
0 (antiglomerular basement membrane antibody)
EC 1.11.1.7 (Peroxidase)

Rapidly progressive glomerulonephritis with pulmonary hemorrhage

Date Created: 20110524 Date Completed: 20111207 Latest Revision: 20211203

20221213

10.3109/0886022X.2011.581401

21599422