Wegener's granulomatosis

Question 1

What is it also known as?

Answer 1

granulomatosis w polyangitis

Question 2

What is it?

Answer 2

ANCA associated vasculitis

necrotising granulomatous inflammation and vasculitis of small and medium vessels

Question 3

What parts of the body can be affected?

Answer 3

Mainly:

1. Upper + lower resp tract
2. Kidneys

Question 4

How can it present?

Answer 4

URT - epistaxis, sinusitis, nasal crusting 
LRT - dyspnoea, haemoptysis
Rapidly progressive glomerulonephritis
Saddle shape nose deformity 
Vasculitic rash

Question 5

What investigations can you do/

Answer 5

cANCA +ve (pANCA in 25%)
CXR
Renal biopsy - epithelial crescents in Bowmans capsule

Question 6

What is the management ?

Answer 6

Steroids
Cyclophosphamide
Plasma exchange