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Anti-Glomerular Basement Membrane Disease

An uncommon, very aggressive type of glomerulonephritis that can cause loss of kidney function over days to weeks.  Also known as Goodpasture's disease.  It may be accompanied by lung haemorrhage which can also be very sudden and life-threatening.  It is caused by autoimmunity to a specific component of specialized basement membranes. 

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This page was last modified on 13/06/2010 at 00:25.

Anti-GBM disease in brief

Introduction and epidemiology - Anti-glomerular basement membrane (anti-GBM) disease is the archetypal aggressive crescentic glomerulonephritis, caused by autoimmunity to a specific basement membrane collagen isoform. The disease is uncommon and mainly seen in caucasian races.  It has a bimodal age incidence with peaks around ages 25 and 55 ...  read moreChest radiographs of a 23-year-old woman who presented with pulmonary haemorrhage (a) 1 week before admission and (b) on admission with a 2-week history of breathlessness and three minor episodes of haemoptysis over 3 months, the last 4 days previously. She had a haemoglobin of 5.4 g/dl with indications of iron deficiency. Her renal biopsy is shown in Fig. 6 ; she had microscopic haematuria, but her serum creatinine was normal at 69 ?mol/l. Circulating anti-GBM antibodies were detected by radioimmunoassay, and direct immunofluorescence of the renal biopsy showed linear fixation of IgG and C3 to the GBM. The radiological changes resolved following treatment with cyclophosphamide, prednisolone, and plasma exchange; but 2 weeks later she had a relapse with pulmonary haemorrhage 1 day after resuming smoking.

Clinical features - The illness is commonly perceived to be relatively minor until rapidly progressive renal failure or lung haemorrhage are recognised.  Isolated renal disease is often recognised late.  Lung disease is largely restricted to smokers, and is often severe at presentation but may be subclinical for some weeks or more previously ... read more.

Investigations:  Biochemical findings are of acute renal failure without any defining features.  Renal biopsy is the most sensitive test although immunoassays can be helfpul.  Lung findings are often non-specific and may be patchy ...  read more

Aetiology and pathogenesis - Antibodies to the Goodpasture antigen, a tissue-specific domain of basement membrane collagen, are of high affinity and have a specific epitope. Genetic predisposition is conferred by HLA-DR15 and other class II MHC alleles may be protective. The disease may be precipitated by things that damage or alter GBM turnover ... read more

Treatment and outcome - Treatment with cyclophosphamide, prednisolone and plasma exchange was developed in the 1970s, and was the first unequivocally effective treatment for an aggressive renal disease.  The treatment was successfully adapted to other types of severe inflammatory glomerulonephritis.   ... read more

Special circumstances - recurrence after transplantation is likely if autoantibodies are still present. A similar disease, Alport anti-GBM disease, occurs rarely in patients whose own kidney fails to express the Goodpasture antigen. 

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