Plasma exchange in anti-glomerular basement membrane disease.

Abstract

Anti-glomerular basement membrane (GBM) disease is a rare autoimmune vasculitis characterised by antibodies directed against the non collagenous (NC1) domain of the α3 chain of type 4 collagen (α3(IV)NC1). Clinical features are typically of a rapidly progressive glomerulonephritis (RPGN) with or without pulmonary haemorrhage. Treatment aims to rapidly remove circulating autoantibodies with plasma exchange and prevent further antibody production and suppress inflammation using immunosuppression and corticosteroids. Retrospective studies have shown that this combination of treatment results in good renal outcomes compared to historical controls. Disease relapse is uncommon and, unless patients have a co-existing antineutrophil cytoplasm antibody, maintenance treatment is not required.

• Authors: Maria Prendecki, Charles Pusey
• Journal: Presse medicale (Paris, France : 1983) (Presse Med) Vol. 48 Issue 11 Pt 2 Pg. 328-337 (Nov 2019) ISSN: 2213-0276 [Electronic] France
• PMID: 31703956 (Publication Type: Journal Article, Review)
• Copyright: Copyright © 2019 Elsevier Masson SAS. All rights reserved.

Chemical References

• Adrenal Cortex Hormones
• Autoantigens
• Collagen Type IV
• Immunosuppressive Agents
• type IV collagen alpha3 chain

Topics

• Adrenal Cortex Hormones (therapeutic use)
• Anti-Glomerular Basement Membrane Disease (complications, diagnosis, immunology, therapy)
• Autoantigens (immunology)
• Biopsy
• Collagen Type IV (immunology)
• Combined Modality Therapy (methods)
• Glomerulonephritis (complications)
• Humans
• Immunosuppressive Agents (therapeutic use)
• Inflammation (drug therapy)
• Kidney (pathology)
• Plasma Exchange
• Retrospective Studies