Granulomatosis with Polyangiitis (Wegener's granulomatosis)

Question 1

What is granulomatosis with polyangiitis?

Answer 1

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

Question 2

What are the features of GPA?

Answer 2

Upper RT: epistaxis, sinusitis, nasal crusting
Lower RT: dyspnoea, haemoptysis

Rapidly progressive glomerulonephritis

Saddle-shaped nose deformity

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

Question 3

What are the investigations for GPA?

Answer 3

cANCA positive (some pANCA positive)
Chest x-ray: various, can have cavitating chest lesions
Renal biopsy: epithelial crescents in bowman's capsule

Question 4

What is the management of GPA?

Answer 4

Steroids
Cyclophosphamide
Plasma exchange
Median survival (8-9 years)