Eosionphilic Granulomatosis with Polyangiitis

Question 1

pathogenesis

Answer 1

Eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels. Associated with asthma

Question 2

who gets it?

Answer 2

associated with asthma

Question 3

how is it classified?

Answer 3

Localized – Upper/Lower respiratory tract disease without any other systemic involvement or constitutional symptomsEarly systemic -any, without organ or life threatening Generalized – renal (creatinine < 500) or other organ threateningSystemic – renal (creatinine > 500) or other vital organ failureRefractory – progressive disease unresponsive steroids + cyclo

Question 4

how does it present?

Answer 4

American College of rheumatology criteria – 4 or more: o asthma: wheezing, expiratory rhonchio eosinophilia of more than 10% in peripheral bloodo paranasal sinusitiso pulmonary infiltrates (may be transient)o histological proof of vasculitis with extravascular eosinophilso mononeuritis multiplex or polyneuropathy

Question 5

how is it investigated

Answer 5

ESR, PV, CRP raisedanaemia of chronic diseaseU&E for renal involvementurinalysis CXRbiopsy of affected area – skin, kidneyimmunology: ANCA