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: Upper Respiratory
    1. Severe Asthma
    2. Allergic Rhinitis
  2. Phase 2: 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

VII. Complications (often cause of death)

  1. Coronary arteritis
  2. Myocarditis

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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