Resp - Granulomatosis with polyangiitis (Wegener's granulomatosis)

Question 1

Granulomatosis with polyangiitis (Wegener’s granulomatosis) or GPA - what is it?

Answer 1

Granulomatosis with polyangiitis is now the preferred term for Wegener’s granulomatosis

Autoimmune condition associated with a necrotizing granulomatous vasculitis, affecting both the upper and lower respiratory tract and kidneys

Question 2

GPA - clinical features of upper and lower respiratory tract, kidneys, and everything else?

Answer 2

Upper respiratory tract: epistaxis, sinusitis, nasal crusting

Lower respiratory tract: dyspnoea, haemoptysis

Kidneys: Rapidly progressive glomerulonephritis (‘pauci-immune’, 80% of patients)

Saddle-shape nose deformity

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

Question 3

GPA - investigations?

Answer 3

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

chest x-ray: cavitating lesions

renal biopsy: epithelial crescents in Bowman’s capsule

Question 4

GPA - management?

Answer 4

steroids

cyclophosphamide (90% response) - suppresses immune system

plasma exchange

median survival = 8-9 years

Question 5

GPA - mnemonic from Passmed, ‘C’s’

Answer 5

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