Granulomatosis with polyangiitis

Question 1

What was the previous term for Granulomatosis with polyangiitis?

Answer 1

Wegener’s granulomatosis

Question 2

What is Granulomatosis with polyangiitis?

Answer 2

multisystem necrotising non-caseating granulomatous vasculitis affecting small to medium-sized vessels, with a predilection for resp system + kidneys.

Question 3

Give 3 presenting symptoms of GPA affecting the upper respiratory tract

Answer 3

Epistaxis
Sinusitis
Nasal crusting

Question 4

Give 2 presenting symptoms of GPA affecting the lower respiratory tract

Answer 4

Dyspnoea
Haemoptysis

Question 5

What does GPA cause in the kidneys?

Answer 5

Rapidly progressive glomerulonephritis
(‘pauci-immune’ in 80%)

Question 6

Give 4 non respiratory/ renal manifestations of GPA

Answer 6

Saddle shaped nose
Vasculitic rash
Eye involvement: Proptosis
CN lesions

Question 7

Which antibody is positive in >90% of patients with GPA?

Answer 7

cANCA

Question 8

Which antibody is positive in ~25% of patients with GPA?

Answer 8

pANCA

Question 9

What may be seen on CXR in GPA?

Answer 9

Cavitating lesions

Question 10

What may be seen on renal biopsy in GPA?

Answer 10

Epithelial crescents in Bowman’s capsule

Question 11

Describe the management of GPA

Answer 11

High dose steroids
Immunosuppressant 1. MTX 2. Cyclophosphamide
Plasma exchange

Question 12

What is the median survival of GPA?

Answer 12

8-9 years