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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

Initial evaluation: as per KDIGO 2021 guidelines, obtain prompt evaluation for Anti-GBM disease in all patients with suspected RPGN.
E
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Diagnostic procedures

Kidney biopsy: as per KDIGO 2021 guidelines, perform kidney biopsy with immunofluorescence at presentation or within 24 hours in patients with suspected RPGN.
E

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.
E
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.
E

More topics in this section

  • 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.
E

Surgical interventions

Kidney transplantation: as per KDIGO 2021 guidelines, postpone kidney transplantation in patients with kidney failure due to Anti-GBM disease until Anti-GBM antibodies remain undetectable for ≥ 6 months.
E

Preventative measures

Prophylaxis for P. jirovecii pneumonia: as per JSN 2016 guidelines, initiate TMP/SMX to improve life prognosis in patients with RPGN receiving with immunosuppressive therapy.
A

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.
E

More topics in this section

  • Management of refractory disease