Anti-GBM disease (Goodpasture’s syndrome)

Anti-GBM disease is a type of small-vessel vasculitis. It is characterised by the presence of autoantibodies against the non-collagenous domain 1 of the α3 chain of type IV collagen (α3(IV)NC1). The target antigen is localised in the glomerular basement membrane of the kidney and in the basement membrane of the pulmonary alveoli. The majority of patients suffer from rapidly progressive glomerulonephritis with micro- or macrohaematuria, usually mild proteinuria and anuria. If the kidneys and lungs are affected (60 – 80 % of patients), it is called Goodpasture's syndrome. In 20 – 40 % of patients with anti-GBM disease, only the kidneys are affected, in a small proportion only the lungs. Untreated, anti-GBM disease can quickly lead to renal failure, lung failure and death. Its incidence is estimated at 0.5 – 1.6 cases per million per year. Anti-GBM autoantibodies are pathogenic: high titers usually occur in patients with rapidly progressive kidney disease. If the antibodies are removed immediately by plasma exchange, the prognosis improves. Relapses are rare if anti-GBM autoantibodies are no longer produced after plasma exchange and under immunosuppression. If anti-GBM autoantibodies can be detected in the blood before a kidney transplant, a relapse in the transplanted kidney is very likely.