title: A case of Goodpasture syndrome positive for anti-GBM antibody and MPO-ANCA complicated by a variety of serious infections.
abstract: A 62-year-old female was admitted to our hospital for investigation of acute progressive renal insufficiency and a systemic inflammatory reaction, despite treatment with several antibiotics. Laboratory data revealed severe renal insufficiency and positive titers for the myeloperoxidase anti-neutrophil cytoplasmic and anti-glomerular basement membrane antibodies. The deterioration of her general status did not allow us to perform the renal biopsy. Although corticosteroid therapy, hemodialysis, and plasma exchange were concomitantly initiated, pulmonary hemorrhage occurred several days after admission. Mechanical ventilation support was provided and continuous hemodiafiltration was carried out, following which the respiratory failure improved immediately. However, she developed clinical depression and suicidal behavior under the intensive therapy. Therefore, plasma exchange was discontinued and corticosteroid was tapered as quickly as possible. Four months after admission, platelet transfusion and short-term mechanical ventilation support improved the pulmonary hemorrhage; however, her mental status deteriorated despite psychiatric consultation and treatment with a tranquilizer. Thereafter, severe and serious systemic infection due to various pathogens including Staphylococcus aureus, Cytomegalovirus, Pneumocystis jiroveci, Pseudomonas aeruginosa, and Bacteroides recurred, and she died from systemic invasive aspergillosis (IA). We suspected severe immunosuppression caused by various factors, such as predonisolone administration, chronic renal failure on maintenance hemodialysis, depression, and malnutrition due to chronic inflammation and granulocytopenia as a side effect of ganciclovir. When treating rapidly progressive glomerulonephritis, immunosuppressive status should be carefully monitored regarding not only the dosage of therapeutic regimen but also the mental health status and nutrition of the patient.
authors.affiliation: Division of Cardiology and Nephrology, Department of Medicine, Kyoto Prefectural University of Medicine, Kyoto, Japan. c-s-1979@koto.kpu-m.ac.jp
authors.forename: C
authors.initials: C
authors.lastname: Sakoda
authors.forename: T
authors.initials: T
authors.lastname: Kusaba
authors.forename: T
authors.initials: T
authors.lastname: Adachi
authors.forename: K
authors.initials: K
authors.lastname: Sonomura
authors.forename: T
authors.initials: T
authors.lastname: Kimura
authors.forename: M
authors.initials: M
authors.lastname: Nakayama
authors.forename: N
authors.initials: N
authors.lastname: Kishimoto
authors.forename: H
authors.initials: H
authors.lastname: Nakagawa
authors.forename: M
authors.initials: M
authors.lastname: Okigaki
authors.forename: T
authors.initials: T
authors.lastname: Hatta
authors.forename: H
authors.initials: H
authors.lastname: Matsubara
authors.forename: Y
authors.initials: Y
authors.lastname: Mori
mesh_terms: D019867:Anti-Glomerular Basement Membrane Disease / Q000150:complications* / Q000276:immunology* / Q000628:therapy; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D001323:Autoantibodies / Q000276:immunology*; D001424:Bacterial Infections / Q000276:immunology*; D017809:Fatal Outcome; D005260:Female; D005938:Glucocorticoids / Q000009:adverse effects / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D044342:Malnutrition / Q000150:complications; D008875:Middle Aged; D010951:Plasma Exchange / Q000009:adverse effects; D011239:Prednisolone / Q000009:adverse effects / Q000627:therapeutic use; D006435:Renal Dialysis / Q000009:adverse effects; D014777:Virus Diseases / Q000276:immunology*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; C051951:antiglomerular basement membrane antibody; D011239:Prednisolone