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Anti-glomerular basement membrane disease
Anti-GBM disease, previously called Goodpasture's disease, is a systemic autoimmune disorder resulting in progressive crescentic glomerulonephritis and, frequently, diffuse pulmonary alveolar hemorrhage.
Anti-GBM disease is caused by an autoimmune response against type IV collagen molecules in the kidneys and lungs triggered by environmental factors in genetically predisposed individuals.
Autoantibodies against type IV collagen molecules in the kidneys and lungs result in anti-GBM disease, which causes clinical manifestations of RPGN with concurrent lung hemorrhage. Disease progression may lead to dialysis-dependent kidney failure, ESRD necessitating kidney transplantation, and respiratory failure.
Prognosis and risk of recurrence
Anti-GBM disease is associated with an in-hospital mortality rate of 7.7 per 100 admissions.
The following summarized guidelines for the evaluation and management of anti-GBM glomerulonephritis are prepared by our editorial team based on guidelines from the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2021) and the Japanese Society of Nephrology (JSN 2016).
Initial evaluation: obtain prompt evaluation forAnti-GBM disease in all patients with suspected RPGN.
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Kidney biopsy: as per KDIGO 2021 guidelines, perform kidney biopsy with immunofluorescence at presentation or within 24 hours in patients with suspected RPGN.
Initiate treatment without delay in patients with suspected anti-GBM disease, even before the diagnosis is confirmed.
Consider offering a conservative approach in patients without alveolar hemorrhage but with oliguria and/or advanced kidney failure requiring dialysis, especially in the presence of a very high proportion of crescents (85-100%) on kidney biopsy.
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As per KDIGO 2021 guidelines:
Initiate plasmapheresis along with immunosuppressive therapy in all patients with anti-GBM glomerulonephritis, except patients receiving dialysis at presentation, having 100% crescents or > 50% global glomerulosclerosis in an adequate biopsy sample, and not having a pulmonary hemorrhage.
Perform plasmapheresis until anti-GBM titers are no longer detectable.
Kidney transplantation: postpone kidney transplantation in patients with kidney failure due toAnti-GBM disease untilAnti-GBM antibodies remain undetectable for ≥ 6 months.
Prophylaxis for Pneumocystis jirovecii pneumonia: initiate TMP/SMX to improve life prognosis in patients with RPGN receiving with immunosuppressive therapy.
7.Follow-up and surveillance
Assessment of treatment response: as per KDIGO 2021 guidelines, monitor kidney function, pulmonary infiltrates,Anti-GBM antibody titers, and blood counts. Modify treatment accordingly.
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Management of refractory disease