Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)

  • “Allergic Granulomatosis”
  • Three clinical phases
    • Prodrome (allergic rhinitis, asthma, eosinophilia)
    • Vasculitis (arthritis, myositis, cardiac, pulmonary, NS, GI, renal, ocular, GU)
    • Post-vasculitic phase (allergic rhinitis, asthma, HTN from renal damage, peripheral nerve damage)
  • Skin lesions: palpable purpura & nodules, retiform purpura, most common in the 2nd phase
 
Course and Treatment
  • Peripheral eosinophilia >10-20%
  • Mononeuritis multiplex is highly associated
  • Leukotriene-inhibitors unmasking - some reports
  • Many cases go undiagnosed until 2nd phase
  • CHF is most common cause of death in the untreated
  • Corticosteroids may be used alone
  • Add cyclophosphamide for refractory cases
  • Maintenance: MTX
  • Rituximab
  • Mepolizumab (anti-IL5 monoclonal antibody)