Goodpasture's syndrome

Question 1

What is Goodpasture’s syndrome?

Answer 1

An autoimmune disease where antibodies attack a type IV collagen found in the basement membrane of the lungs and the kidneys

Question 2

What is the pathophysiology of Goodpasture’s syndrome?

Answer 2

The anti-glomerular basement membrane antibodies lead to small vessel vasculitis, which causes bleeding in the lungs, and renal failure

Question 3

What is the presentation of Goodpasture’s syndrome?

Answer 3

Haemoptysis
Cough
Shortness of breath
Nausea and vomiting
Chest pain
Decreased urine output
Fatigue and malaise
Haematuria

Question 4

What is the definitive investigation for diagnosis of Goodpasture’s syndrome?

Answer 4

Renal biopsy

Question 5

What other investigations are useful in the diagnosis of Goodpasture’s syndrome?

Answer 5

Anti-GBM antibody titre
ANCA antibodies
U&Es
Urinalysis
Chest imaging

Question 6

What is seen on CXR and CT in Goodpasture’s syndrome?

Answer 6

XR - diffuse opacities
CT - ground glass opacities

Question 7

What are the differentials of Goodpasture’s syndrome?

Answer 7

SLE
Granulomatosis with polyangiitis
Anti-GBM disease

Question 8

What are the differences between Goodpasture’s syndrome and Anti-GBM disease?

Answer 8

No lung involvement in anti-GBM disease

Question 9

What is the acute management of Goodpasture’s syndrome?

Answer 9

Plasmapheresis
Prednisolone - tapered over 3 months
Cyclophosphamide

Question 10

What is the long term management of Goodpasture’s syndrome?

Answer 10

Maintenance therapy
- Azathioprine for immune suppression
- Low dose prednisolone
Smoking cessation

Question 11

What are the complications of Goodpasture’s syndrome?

Answer 11

Pulmonary haemorrhage
Plasmapheresis related bleeding
Cyclophosphamide related neutropenia
Immunosuppression related infected
CKD
Long term steroid complications