ILD

Eosinophilic Granulomatosis with Polyangiitis

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Eosinophilic Granulomatosis with Polyangiitis, EGPA, Churg-Strauss Syndrome, Churg-Strauss, Allergic Granulomatosis

  • Epidemiology
  1. Rare disease seen most often in men
  • Pathophysiology
  1. Similar to Classic Polyarteritis Nodosa
  2. Small Vessel, Granulomatous Vasculitis
    1. Multiple organ system involvement
    2. Interstitial Lung Disease
  • Phases
  1. Phase 1: Prodromal (esp. Upper Respiratory)
    1. Severe Asthma
    2. Allergic Rhinitis
    3. Eczema
  2. Phase 2: Eosinophilic (esp. Lower Respiratory)
    1. Eosinophilic lung infiltrate
  3. Phase 3: Vasculitis (occurs within 3 years of onset)
    1. Granulomatous ANCA-associated Small Vessel Vasculitis
  1. Eosinophilic tissue infiltration
  • Labs
  • Complications (often cause of death)
  1. Coronary arteritis
  2. Myocarditis
  • Management
  • References
  1. Calabrese in Ruddy (2001) Kelly's Rheum, p. 1179-81
  2. Eustace (1999) J Am Soc Nephrol 10(9):2048-55 [PubMed]