Goodpasture's disease and Wegener's granulomatosis

Question 1

If goodpasture’s syndrome has auto-antibodies against glomerular basement membrane (Anti-GBM disease), why does the person get haemoptysis?

Answer 1

The auto-antibodies are for type 4 collagen which is found in the glomerular basement membrane AND alveolar capillary BM

Question 2

What is the treatment for goofpasture’s disease?

Answer 2

Plasmapheresis + immunosuppression

Question 3

What auto-antibody is found in Wegener’s granulomatosis and what is it specific for?

Answer 3

c-ANCA > proteinase 3

Question 4

How does methotrexate work?

Answer 4

inhibits metabolism of folic acid (folic acid reductase) > preventing DNA synthesis + cell proliferation

Question 5

What tests should you carry out to Ix Wegener’s?

Answer 5

CXR, Urinalysis, renal biopsy, CT chest, serology (for auto-antibodies)

Question 6

What is the treatment plan for Wegener’s granulomatosis?

Answer 6

1. Prednisolone + cyclophosphamide
2. rituximab
3. Maintain: methotrexate + azathioprine

Question 7

How would you treat severe Wegener’s?

Answer 7

Plasmapheresis.

Question 8

What prophylactic treatment should you give? Why?

Answer 8

Co-trimoxazole to prevent pneumocystis jirovecii infection

Question 9

Exam: What is the differential diagnosis for haemoptysis?

Answer 9

TB, goodpasture’s syndrome, Wegener’s granulomatosis, BRONCHIAL CARCINOMA, bronchiectasis, pulmonary infarction, trauma