Goodpasture's syndrome

Question 1

Goodpasture’s syndrome?

Answer 1

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.

Question 2

Features?

Answer 2

• pulmonary haemorrhage
• followed by rapidly progressive glomerulonephritis

Question 3

Factors which increase likelihood of pulmonary haemorrhage?

Answer 3

• smoking
• lower respiratory tract infection
• pulmonary oedema
• inhalation of hydrocarbons
• young males

Question 4

Investigations?

Answer 4

• renal biopsy: linear IgG deposits along basement membrane
• raised transfer factor secondary to pulmonary haemorrhages

Question 5

Management?

Answer 5

• plasma exchange (plasmapheresis)
• steroids
• cyclophosphamide