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Antiglomerular Basement Membrane Disease.

Stephen P McAdoo¹, Charles D Pusey¹

¹Department of Medicine, Centre for Inflammatory Disease, Imperial College London, London, United Kingdom.

Seminars in Respiratory and Critical Care Medicine

|November 8, 2018

Summary

This summary is machine-generated.

Antiglomerular basement membrane (anti-GBM) disease is a rare autoimmune vasculitis. Prompt treatment with plasmapheresis, cyclophosphamide, and steroids improves kidney function and reduces respiratory sequelae.

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Area of Science:

Nephrology
Rheumatology
Pulmonology

Background:

Antiglomerular basement membrane (anti-GBM) disease is a rare, life-threatening autoimmune vasculitis.
Characterized by autoantibodies targeting type IV collagen in glomerular and alveolar basement membranes.
Manifests as rapidly progressive glomerulonephritis (GN) and/or alveolar hemorrhage.

Purpose of the Study:

To summarize the key aspects of anti-GBM disease.
To highlight diagnostic methods and treatment strategies.
To discuss patient prognosis and relapse factors.

Main Methods:

Review of clinical presentation, serological testing, and renal biopsy findings.
Discussion of treatment protocols including plasmapheresis, cyclophosphamide, and steroids.
Analysis of outcomes, including renal function and respiratory sequelae, and relapse predictors.

Main Results:

Anti-GBM disease typically presents as GN (80-90%) with concurrent alveolar hemorrhage (50%).
Diagnosis is aided by serology and confirmed by renal biopsy showing necrotizing/crescentic GN and linear antibody deposition.
Aggressive treatment maintains independent renal function in >80% of patients at 1 year if not requiring dialysis at presentation.

Conclusions:

Prompt treatment is crucial for managing anti-GBM disease, improving renal and respiratory outcomes.
Relapse is uncommon unless concomitant antineutrophil cytoplasm antibodies are present, necessitating maintenance immunosuppression.
Anti-GBM disease requires a multidisciplinary approach for optimal patient management and prognosis.