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Churg-Strauss Syndrome
Aka: Churg-Strauss Syndrome, Churg-Strauss, Allergic Granulomatosis
- Epidemiology
- Rare disease seen most often in men
- Pathophysiology
- Similar to Classic Polyarteritis Nodosa
- Small Vessel, Granulomatous Vasculitis
- Multiple organ system involvement
- Interstitial Lung Disease
- Phases
- Phase 1: Upper Respiratory
- Severe Asthma
- Allergic Rhinitis
- Phase 2: Lower Respiratory
- Eosinophilic lung infiltrate
- Phase 3: Vasculitis (occurs within 3 years of onset)
- Granulomatous ANCA-associated Small Vessel Vasculitis
- Signs: Interstitial Lung Disease
- Eosinophilic tissue infiltration
- Labs
- Antineutrophil Cytoplasmic Antibody (ANCA)
- Positive in 70% of cases (usually MPO-ANCA)
- Complete Blood Count (CBC)
- Eosinophilia >10%
- Complications (often cause of death)
- Coronary arteritis
- Myocarditis
- Differential Diagnosis
- Polyarteritis Nodosa (medium vessel Vasculitis)
- ANCA-Associated Small Vessel Vasculitis
- Granulomatosis with Polyangiitis (previously known as Wegener's Granulomatosis)
- Microscopic Polyangiitis
- Management
- High-dose Corticosteroids
- Control vascular injury
- Hypertension
- Diabetes Mellitus
- Tobacco abuse
- Hyperlipidemia
- References
- Calabrese in Ruddy (2001) Kelly's Rheum, p. 1179-81
- Eustace (1999) J Am Soc Nephrol 10(9):2048-55 [PubMed]