Eosinophilic Granulomatosis with Polyangiitis

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

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II. Epidemiology

  1. Rare disease seen most often in men

III. Pathophysiology

  1. Similar to Classic Polyarteritis Nodosa
  2. Small Vessel, Granulomatous Vasculitis
    1. Multiple organ system involvement
    2. Interstitial Lung Disease

IV. 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

V. Signs: Interstitial Lung Disease

  1. Eosinophilic tissue infiltration

VI. Labs

  1. Antineutrophil Cytoplasmic Antibody (ANCA)
    1. Positive in 70% of cases (usually MPO-ANCA)
  2. Complete Blood Count (CBC)
    1. Eosinophilia >10%

VII. Complications (often cause of death)

  1. Coronary arteritis
  2. Myocarditis

VIII. Differential Diagnosis

  1. Polyarteritis Nodosa (medium vessel Vasculitis)
  2. ANCA-Associated Small Vessel Vasculitis
    1. Granulomatosis with Polyangiitis (previously known as Wegener's Granulomatosis)
    2. Microscopic Polyangiitis

IX. Management

  1. High-dose Corticosteroids
  2. Control vascular injury
    1. Hypertension
    2. Diabetes Mellitus
    3. Tobacco Abuse
    4. Hyperlipidemia

X. References

  1. Calabrese in Ruddy (2001) Kelly's Rheum, p. 1179-81
  2. Eustace (1999) J Am Soc Nephrol 10(9):2048-55 [PubMed]

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