GOODPASTURES-SYNDROME - NEW INSIGHTS INTO PATHOGENESIS AND CLINICAL PICTURE

Goodpasture's disease is a disorder in which glomerulonephritis and lu ng haemorrhage are associated with autoimmunity against a tissue speci fic component of basement membrane. The defining feature is the presen ce of anti glomerular basement membrane (anti-GEM) antibodies in the c irculation and bound to the glomerular basement membrane, Several patt erns of clinical disease are recognised. An important feature is rapid accelerations in disease progression in which renal function may be p ermanently lost within days or weeks. Profound immunosuppression is cu rrently the only effective treatment of progressive disease, However, understanding of immunopathogenesis is well advanced compared to other forms of human autoimmune glomerulonephritis.