Churg-Strauss Syndrome

 

Epidemiology:

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Common sites:

    • lung
    • heart
    • spleen
    • peripheral nerves
    • skin

 

Gross features:

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Histologic features:

    • necrotizing vasculitis accompanied by granulomas with eosinophilic necrosis
      • intravascular and extravascular granulomas
    • striking infiltration of vessels and perivascular tissues by eosinophils
    • may have similar vascular lesions to PAN or microscopic polyangiitis
    • early prevasculitic phase:
      • tissue infiltration by eosinophils
      • no overt vasculitis

 

Immunophenotype:

Marker:

Sensitivity:

Specificity:

 

 

 

 

Molecular features:

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Other features:

    • p-ANCAs in 50%
    • strong association to:
      • allergic rhinitis
      • asthma
      • eosinophilia
    • coronary arteritis and myocarditis are principal cause of morbidity and mortality

 

References:

    • Robbins 2005