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ALL / Nephrology
Anti-glomerular basement membrane (GBM) disease (Goodpasture's syndrome)
Anti-glomerular basement membrane (GBM) disease (Goodpasture's syndrome)
Anti-glomerular basement membrane (GBM) disease (previously known as Goodpasture's syndrome) is a rare type of small-vessel vasculitis associated with both pulmonary haemorrhage and rapidly progressive glomerulonephritis. It is caused by anti-glomerular basement membrane (anti-GBM) antibodies against type IV collagen. Goodpasture's syndrome is more common in men (sex ratio 2:1) and has a bimodal age distribution (peaks in 20-30 and 60-70 age bracket). It is associated with HLA DR2.Features
- pulmonary haemorrhage
- rapidly progressive glomerulonephritis
- this typically results in a rapid onset acute kidney injury
- nephritis → proteinuria + haematuria
Investigations
- renal biopsy: linear IgG deposits along the basement membrane
- raised transfer factor secondary to pulmonary haemorrhages
Management
- plasma exchange (plasmapheresis)
- steroids
- cyclophosphamide
One of the main complications is pulmonary haemorrhage. Factors that increase the likelihood of this include:
- smoking
- lower respiratory tract infection
- pulmonary oedema
- inhalation of hydrocarbons
- young males
+PassMedicine Notes
Anti-glomerular basement membrane (GBM) disease (previously known as Goodpasture's syndrome) is a rare type of small-vessel vasculitis associated with both pulmonary haemorrhage and rapidly progressive glomerulonephritis. It is caused by anti-glomerular basement membrane (anti-GBM) antibodies against type IV collagen. Goodpasture's syndrome is more common in men (sex ratio 2:1) and has a bimodal age distribution (peaks in 20-30 and 60-70 age bracket). It is associated with HLA DR2.
Features- pulmonary haemorrhage
- rapidly progressive glomerulonephritis
- this typically results in a rapid onset acute kidney injury
- nephritis --> proteinuria + haematuria
- renal biopsy: linear IgG deposits along the basement membrane
- raised transfer factor secondary to pulmonary haemorrhages
- plasma exchange (plasmapheresis)
- steroids
- cyclophosphamide
- smoking
- lower respiratory tract infection
- pulmonary oedema
- inhalation of hydrocarbons
- young males