Eosinophilic granulomatosis with polyangiitis



Introduction
Epidemiology
Aetiology
Clinical features
Differential diagnosis


Introduction

Eosinophilic granulomatosis with polyangiitis (EGPA) is now the preferred term for Churg-Strauss syndrome. It is an ANCA associated small-medium vessel vasculitis.

Epidemiology

  • Incidence: 0.20 cases per 100,000 person-years
  • Peak incidence: 40-50 years
  • Sex ratio: 1:1
Condition Relative
incidence
Granulomatosis with polyangiitis5.00
Eosinophilic granulomatosis with polyangiitis1
Goodpasture's syndrome0.50
<1 1-5 6+ 16+ 30+ 40+ 50+ 60+ 70+ 80+

Aetiology

Leukotriene receptor antagonists may precipitate the disease.

Clinical features

Features
  • asthma
  • blood eosinophilia (e.g. > 10%)
  • paranasal sinusitis
  • mononeuritis multiplex
  • pANCA positive in 60%

Differential diagnosis

Comparison of granulomatosis with polyangiitis and Churg-Strauss syndrome