Goodpasture’s syndrome (anti‑GBM disease)
Overview
Goodpasture’s syndrome (anti‑glomerular basement membrane, anti‑GBM, disease) is a rare, often rapidly progressive autoimmune small‑vessel vasculitis that classically produces a pulmonary-renal syndrome: diffuse alveolar haemorrhage (DAH), rapidly progressive glomerulonephritis (RPGN) and circulating anti‑GBM antibodies. Early recognition in the emergency department is critical because prompt plasmapheresis and immunosuppression can be lifesaving and may preserve renal function.
Epidemiology and risk factors
- Rare condition with a male predominance.
- Bimodal age distribution is commonly described (young adults and older adults).
- Smoking predisposes to pulmonary haemorrhage.
- Associated with HLA‑B7 and HLA‑DR2.
- About 25-30% of patients are “double‑positive” for anti‑GBM and ANCA; this affects presentation and management.
Pathophysiology
Autoantibodies target the α3 chain of type IV collagen in glomerular and alveolar basement membranes, causing complement activation, neutrophil recruitment and capillary injury. In the kidney this produces crescentic necrotising glomerulonephritis; in the lung this produces DAH with alveolar filling and impaired gas exchange.
Presentation and clinical features
Onset ranges from subacute to fulminant. Actively assess for both pulmonary and renal involvement.