Goodpastures Syndrome

Goodpasture’s syndrome is a disease of unknown origin characterised by a pulmonary renal syndrome which include haemorrhage, glomerulonephritis and the presence of circulating antibodies against basement membrane antigens. Renal involvement can rapidly progress causing failure within a short timeframe from onset of disease. This can result in permanent renal failure.

A major autoantigen associated with Goodpasture’s is the non-collagenous domain 1 of collagen type IV. Antigens are generally purified from the kidney and work well as long as they are produced in eukaryotic systems. Anti-GBM antibodies have a prognostic value depending on their titre which can directly reflect severity. Antibodies to one of the two identified major epitopes causes toxicity and progression of the disease. Many patients with anti-GBM antibodies also have ANCA with specificity for MPO being the most common. 

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