GPA- Wegener's disease

Question 1

GPA- full form

Answer 1

Granulomatosis with polangiitis- wegener’s granulomatosis

Question 2

Features of GPA

Answer 2

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 3

GPA and Churg-Strauss syndrome similarities

Answer 3

BOTH present with Vasculitis, sinusitis and dyspnoea

Question 4

How is Churg-Strauss different to GPA

Answer 4

Asthma, pANCA, Eosinophilia

Question 5

GPA- specific symptoms and signs- differences between Churg-Strauss

Answer 5

Renal Failure

Epistasix, haemoptysis, cANCA

Question 6

Investigations of GPA

Answer 6

cANCA positive in > 90%, pANCA positive in 25%
chest x-ray: wide variety of presentations, including cavitating lesions- cavities of AIR
renal biopsy: epithelial crescents in Bowman’s capsule

Question 7

Management for GPA

Answer 7

-STeroids
-Cyclophosamide
Plasma exchange
Median-survival - 8-9 years