Pulmonology Book


Churg-Strauss Syndrome

Aka: Churg-Strauss Syndrome, Churg-Strauss, Allergic Granulomatosis
  1. Epidemiology
    1. Rare disease seen most often in men
  2. Pathophysiology
    1. Similar to Classic Polyarteritis Nodosa
    2. Small Vessel, Granulomatous Vasculitis
      1. Multiple organ system involvement
      2. Interstitial Lung Disease
  3. 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
  4. Signs: Interstitial Lung Disease
    1. Eosinophilic tissue infiltration
  5. Labs
    1. Antineutrophil Cytoplasmic Antibody (ANCA)
      1. Positive in 70% of cases (usually MPO-ANCA)
    2. Complete Blood Count (CBC)
      1. Eosinophilia >10%
  6. Complications (often cause of death)
    1. Coronary arteritis
    2. Myocarditis
  7. 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
  8. Management
    1. High-dose Corticosteroids
    2. Control vascular injury
      1. Hypertension
      2. Diabetes Mellitus
      3. Tobacco Abuse
      4. Hyperlipidemia
  9. 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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