Respiratory Medicine - Granulomatosis with polyangiitis (Wegener's granulomatosis)

Question 1

What is Granulomatosis with polyangiitis (Wegener’s granulomatosis)? (preferred term for Wegener’s granulomatosis)

Answer 1

1. It is an autoimmune condition associated with a necrotizing granulomatous vasculitis,
2. Affecting both the upper and lower respiratory tract as well as the kidneys.

Question 2

Features of Granulomatosis with polyangiitis?

Answer 2

1. Upper respiratory tract: epistaxis, sinusitis, nasal crusting
2. Lower respiratory tract: dyspnoea, haemoptysis
3. Rapidly progressive glomerulonephritis (‘pauci-immune’, 80% of patients)
4. Saddle-shape nose deformity
5. Also: vasculitic rash, eye involvement (e.g. proptosis), cranial nerve lesions

Question 3

Investigations?

Answer 3

1. cANCA positive in > 90%, pANCA positive in 25%
2. Chest x-ray: wide variety of presentations, including cavitating lesions
3. Renal biopsy: epithelial crescents in Bowman’s capsule

Question 4

Management?

Answer 4

1. Steroids
2. Cyclophosphamide (90% response)
3. Plasma exchange
4. Median survival = 8-9 years