Granulomatosis with polyangiitis Flashcards
1
Q
What is Granulomatosis with polyangiitis also known as?
A
Wegener’s granulomatosis
2
Q
What is GPA?
A
autoimmune condition associated with a necrotizing granulomatous vasculitis, affecting upper and lower respiratory tract as well as the kidneys
3
Q
Clinical features of GPA
A
upper respiratory tract:
- epistaxis
- sinusitis
- nasal crusting
lower respiratory tract:
- dyspnoea
- haemoptysis
- rapidly progressive glomerulonephritis
- saddle-shape nose deformity
- vasculitic rash
- eye involvement (e.g. proptosis)
- cranial nerve lesions
4
Q
investigation findings in GPA
A
cANCA positive
CXR: may have cavitating lesions
Renal biopsy: epithelial crescents in Bowman’s capsule
5
Q
Management of GPA
A
- steroids
- cyclophosphamide
- plasma exchange
6
Q
Median survival in GPA
A
8-9 years
