Small vessel vasculitis - GOODPASTURE'S SYNDROME (Anti-glomerular basement membrane disease)

Question 1

What is it?

Answer 1

A rare condition causing inflammation of small blood vessels in the kidneys & lungs.

Question 2

what is the characteristic triad of the disease?

Answer 2

1) pulmonary haemorrhage
= acute bleeding from the lungs

2) glomerulonephritis
= acute inflammation of the kidney

3) presence of circulating antibodies
- detected by antigen intrinsic to basement membrane of both kidney & lung

Question 3

what are the symptoms?

Answer 3

respiratory symptom often precede the onset of the disease.

• haemoptysis
• cough
• shortness of breath
• dyspnoea
• chest pain

Question 4

what would a chest X-ray show?

Answer 4

• transient patchy shadows due to intrapulmonary haemorrhage

Question 5

what effect would the disease have on carbon monoxide transfer?

Answer 5

it would increase due to haemoglobin in the alveoli

Question 6

how would you treat it?

Answer 6

1) treat it with plasmapheresis

2) immunosuppression (prednisolone & fyclophamide)

Question 7

why would you treat it with plasmapheresis?

Answer 7

to remove circulating antibodies

Question 8

why would you give immunosupppresion drugs?

Answer 8

to prevent further antibody production