Goodpasture syndrome

- occurs in people age more than 16 (male:female = 6:1)
- type II cytotoxic reaction (antibodies against basement membrane of both kidney and lungs)
- 80% HLA BR2 positive
- triad of glomerulonephritis, pulmonary hemorrhage and anti-GBM positive (against alpha-3 chain of type IV collagen) *alpha-5 in Alport's syndrome
- starts with URTI symptoms followed by cough, hemoptysis, tiredness and anemia.
- acute glomerulonephritis may presents with asymptomatic proteinuria or microscopic hematuria
- chest X-ray: bilateral diffuse infiltrates in lower zone, transient blotchy shadows (intrapulmonary hemorrhage)
- diffusion capacity for carbon monoxide is increased (pulmonary hemorrhage)
- diagnosis: renal biopsy (gold standard) - crescentic nephritis
- serial TLCO measurement can be used to monitor progresssion
- ANCA may be positive
- treatment: corticosteroid, cyclophosphamide, plasmapheresis