Journal
THERAPEUTIC APHERESIS AND DIALYSIS
Volume 26, Issue 1, Pages 24-31Publisher
WILEY
DOI: 10.1111/1744-9987.13718
Keywords
anti-GBM disease; Goodpasture; IdeS; plasma exchange; rituximab
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Anti-glomerular basement membrane (GBM) disease often manifests as severe kidney failure and pulmonary hemorrhage, with some patients having a more severe disease phenotype and poor prognosis. Treatment with therapeutic plasma exchange, cyclophosphamide, and glucocorticoids is standard, but additional developments are necessary to refine treatment approaches.
Anti-glomerular basement membrane (GBM) disease (Goodpasture disease) often presents with severe kidney failure and pulmonary hemorrhage. Anti-GBM antibodies are pathogenic, and other autoantibodies such as laminin-521 have been identified recently, potentially indicating a subset with a more severe disease phenotype and poor prognosis. Around 30%-40% of patients are also anti-neutrophil cytoplasmatic antibody (ANCA)-positive and this subset combines features of anti-GBM disease and ANCA-associated vasculitis, with particular impact on long-term treatment. A combination of therapeutic plasma exchange (or immunoadsorption), cyclophosphamide, and glucocorticoids is considered standard of care management, but despite early initiation, patients with poor prognostic factors often remain dialysis dependent. Imlifidase (IdeS), capable to cleave IgG within hours, has been tested in a phase II trial. Among 15 patients, 10 with poor prognosis at baseline (eGFR <15 ml/min/1.73 m(2)) were dialysis independent at 6 months. Further developments are needed to refine treatment approaches in anti-GBM disease.
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