Reviews
Goodpasture syndrome and anti-glomerular basement membrane disease
F. Reggiani1, V. L'imperio2, M. Calatroni3, F. Pagni4, R.A. Sinico5
- Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Milan, and Nephrology and Dialysis Unit, IRCCS Humanitas Research Hospital, Rozzano, Milan, Italy.
- Department of Medicine and Surgery, Pathology, University of Milano-Bicocca, IRCCS (Scientific Institute for Research, Hospitalisation and Healthcare) Fondazione San Gerardo dei Tintori, Monza, Italy.
- Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Milan, and Nephrology and Dialysis Unit, IRCCS Humanitas Research Hospital, Rozzano, Milan, Italy.
- Department of Medicine and Surgery, Pathology, University of Milano-Bicocca, IRCCS (Scientific Institute for Research, Hospitalisation and Healthcare) Fondazione San Gerardo dei Tintori, Monza, Italy.
- Nephrology and Dialysis Unit, IRCCS Humanitas Research Hospital, Rozzano, Milan, Italy. renato.sinico@humanitas.it
CER16676
2023 Vol.41, N°4
PI 0964, PF 0974
Reviews
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PMID: 36995324 [PubMed]
Received: 15/03/2023
Accepted : 20/03/2023
In Press: 30/03/2023
Published: 18/04/2023
Abstract
Anti-glomerular basement membrane (anti-GBM) disease is a rare life-threatening small vessel vasculitis that typically affects the capillaries of kidneys and lungs, with most of patients developing rapidly progressive crescentic glomerulonephritis, and 40%-60% concomitant alveolar haemorrhage. It is caused by the deposition in alveolar and glomerular basement membrane of circulating autoantibodies directed against antigens intrinsic to the basement membrane. The exact mechanism that induces the formation of autoantibodies is unknown, but probably environmental factors, infections or direct damage to kidneys and lungs may trigger the autoimmune response in genetically susceptible individuals. Initial therapy includes corticosteroids and cyclophosphamide to prevent autoantibodies production, and plasmapheresis to remove the circulating autoantibodies. Good renal outcomes may be achieved by a prompt treatment initiation. However, when patients present with severe renal failure requiring dialysis or with a high proportion of glomerular crescents at biopsy, renal outcomes are bad. Relapses are rare and when renal involvement is present, the suspect of concomitant diseases, such as ANCA-associated vasculitis and membranous nephropathy, should be raised. Imlifidase is showing promising results, which if confirmed will cause a paradigm shift in the treatment of this disease.
