GPS (Good-Pasture’-Syndrome)
GPS 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
· 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
· steroids
· cyclophosphamide
