Good Pasture’s Disease

Question 1

What is Goodpasture’s?

Answer 1

X-linked condition: autoantibodies mainly IgG that target type IV collagen, which is present in both the glomerular and alveolar basement membranes causing Type II hypersensitivity

Question 2

How does Anti-GM present?

Answer 2

Presentation is with haematuria, rapidly progressive renal impairment and pulmonary haemorrhage, which may lead to respiratory failure

Crackles on lower zones of lungs
Cyanosis

Question 3

What are the investigations for anti-GBM?

Answer 3

Investigations include urinalysis, blood tests including anti-GBM antibodies, chest X-ray, renal ultrasound and renal biopsy

Question 4

What is the initial management of anti-GBM?

Answer 4

Plasma exchange to remove circulating antibodies form blood

Question 5

How does anti-GBM affect kidneys?

Answer 5

Causes rapidly progressive glomerulonephirits

Question 6

How does anti-GBM AFFECT lungs?

Answer 6

A necrotising haemorrhagic interstitial pneumonitis occurs in the lungs

Question 7

What are risk factors for anti-GBM?

Answer 7

Smoking
Exposure to hydrocarbons and metal dust
Infection
Alemtuzumab monoclonal antibody

Question 8

What does CXR show for Good pastures?

Answer 8

Chest X-ray shows widespread airway opacities in pulmonary haemorrhage (may appear identical to pulmonary oedema)

Question 9

What does CT chest show for Goodpasture’s?

Answer 9

CT chest typically shows ground glass and airspace opacities, progressing to a “crazy paving” appearance with septal thickening over the following weeks

Question 10

What is the gold standard for diagnosis of anti-GBM?

Answer 10

Renal biopsy is the gold standard for diagnosis, demonstrating linear IgG deposition along the basement membrane

Question 11

What is the initial management of anti-GBM?

Answer 11

Plasmapheresis with immunosuppressive medication until anti-GBM antibodies are no longer detectable

Question 12

WHA

Question 13

What is the msst common cause fo death in anti-GBM?

Answer 13

Respiratory failure secondary to pulmonary haemorrhage

Question 14

Which occupation is a risk factor for anti-GBM?

Answer 14

Exposure to solvents

Question 15

What is a marker of pulmonary haemorrhage in Goodpasture’s syndrome?

Answer 15

Raised transfer factor

Question 16

What is a risk for Goodpasture’s?

Answer 16

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