Goodpasture Syndrome

Overview

A rare autoimmune disease in which antibodies attack collagen antigens in the lung and kidney basement membrane (anti-glomerular basement membrane disease), causing haemoptysis and haematuria. Presents with cough, malaise, fevers, and weight loss. Age of onset is bimodal — 20–30 and over 60.

Risk factors: anything damaging collagen in the lungs/kidneys and exposing the alpha-3 chain antigen, smoking, infection, hydrocarbon-based solvent exposure (e.g. dry cleaning).

Consider in a patient with haemoptysis plus decreased renal function or haematuria. A rare cause of restrictive lung disease.

Diagnosis

Requires tissue biopsy, usually renal. Antibodies may be detectable for a few weeks to 2 years, after which the disease usually resolves spontaneously.

Prognosis: ~80% 5-year survival; ~30% require dialysis.

Treatment

Steroids and plasmapheresis, now often combined with rituximab as an additional immunosuppressive agent.