Anti-glomerular Basement Membrane (GBM) Disease
Anti-GBM a rare autoimmune disorder in which antibodies are directed against an antigen normally present in the GBM and alveolar basement membrane, specifically the alpha-3 chain of type IV collagen
The disease has a bimodal age presentation at 3rd decade (usually males affected) and 6th-7th decade
Presentation
- 80-90% present with RPGN, 40-60% diffuse alveolar haemorrhage (DAH), <10% isolated pulmonary disease
Diagnosis
- positive anti-GBM (serology or tissue)
- RPGN + / - DAH
Treatment
- Early and aggressive plasma exchange
- Immunesuppression (high steroids and alkylating agent or B cell depletion)
