Granulomatosis with polyangiitis

Question 1

Features of Granulomatosis with Polyangiitis

Answer 1

upper respiratory tract: epistaxis, sinusitis, nasal crusting

lower respiratory tract: dyspnoea, haemoptysis

rapidly progressive glomerulonephritis (‘pauci-immune’, 80% of patients)

saddle-shape nose deformity

also: vasculitic rash, eye involvement (e.g. proptosis), cranial nerve lesions

Question 2

Investigations of Granulomatosis with Polyangiitis

Answer 2

cANCA positive in > 90%, pANCA positive in 25%

chest x-ray: wide variety of presentations, including cavitating lesions

renal biopsy: epithelial crescents in Bowman’s capsule

Question 3

Management of Granulomatosis with Polyangiitis

Answer 3

steroids

cyclophosphamide (90% response)

plasma exchange

median survival = 8-9 years

Question 4

Wegener’s: I C your nose bleeding!

What are the C’s?

Answer 4

Wegener’s: I C your nose bleeding!
C-ANCA
C shaped saddle nose
Crusting (nasal), epistaxis
Cranial nerve lesions
Crescents (epithelial) in Bowman’s capsule - ‘pauci-immune’ RPGN
Cyclophosphamides and steroids
Cavitating lesions/nodules with Central lucency on CXR.
Cinusitis/vasculitic rash