Table of contents
Anti-glomerular basement membrane disease
What's new
Updated 2021 KDIGO and 2016 JSN guidelines for the diagnosis and management of anti-glomerular basement membrane disease.
Background
Overview
Definition
Anti-GBM disease, previously called Goodpasture's disease, is a systemic autoimmune disorder resulting in progressive crescentic glomerulonephritis and, frequently, diffuse pulmonary alveolar hemorrhage.
1
Pathophysiology
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.
2
Disease course
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.
2
Prognosis and risk of recurrence
Anti-GBM disease is associated with an in-hospital mortality rate of 7.7 per 100 admissions.
3
Guidelines
Key sources
The following summarized guidelines for the evaluation and management of anti-glomerular basement membrane disease 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)....
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Diagnostic investigations
Diagnostic procedures
Medical management
General principles
As per KDIGO 2021 guidelines:
Initiate treatment without delay in patients with suspected anti-GBM disease, even before the diagnosis is confirmed.
B
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.
C
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Immunosuppressive therapy
IVIG
Maintenance therapy
Antithrombotic therapy
Therapeutic procedures
Plasmapheresis
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.
B
Perform plasmapheresis until anti-GBM titers are no longer detectable.
B