Goodpasture's syndrome
Goodpasture's syndrome is rare condition 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
Factors which increase likelihood of pulmonary haemorrhage
Investigations
Management
Features
- pulmonary haemorrhage
- followed by rapidly progressive glomerulonephritis
Factors which increase likelihood of pulmonary haemorrhage
- smoking
- lower respiratory tract infection
- pulmonary oedema
- inhalation of hydrocarbons
- young males
Investigations
- renal biopsy: linear IgG deposits along basement membrane
- raised transfer factor secondary to pulmonary haemorrhages
Management
- plasma exchange (plasmapheresis)
- steroids
- cyclophosphamide