The American College of Rheumatology 1990 criteria for the classification of churg-strauss syndrome (allergic granulomatosis and angiitis)

Authors

  • Alfonse T. Masi MD, DRPH,

    Corresponding author
    1. University of Illinois College of Medicine, Peoria, IL
    • American College of Rheumatology, 17 Executive Park Drive NE, Atlanta, GA 30329
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  • Gene G. Hunder MD,

    Chair, Corresponding author
    1. Mayo Clinic, Rochester, MN Subcommittee on Classification of Vasculitis
    • American College of Rheumatology, 17 Executive Park Drive NE, Atlanta, GA 30329
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  • J. T. Lie MD,

    Corresponding author
    1. Mayo Clinic, Rochester, MN
    • American College of Rheumatology, 17 Executive Park Drive NE, Atlanta, GA 30329
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  • Beat A. Michel MD,

    Corresponding author
    1. Rheumaklinik Universitätsspital, Zurich, Switzerland
    • American College of Rheumatology, 17 Executive Park Drive NE, Atlanta, GA 30329
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  • Daniel A. Bloch PhD,

    Corresponding author
    1. Stanford University, Stanford, CA
    • American College of Rheumatology, 17 Executive Park Drive NE, Atlanta, GA 30329
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  • William P. Arend MD,

    Corresponding author
    1. University of Colorado Health Science Center, Denver, CO
    • American College of Rheumatology, 17 Executive Park Drive NE, Atlanta, GA 30329
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  • Leonard H. Calabrese DO,

    Corresponding author
    1. Cleveland Clinic Foundation, Cleveland, OH
    • American College of Rheumatology, 17 Executive Park Drive NE, Atlanta, GA 30329
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  • Steven M. Edworthy MD,

    Corresponding author
    1. University of Calgary, Calgary, Alberta, Canada
    • American College of Rheumatology, 17 Executive Park Drive NE, Atlanta, GA 30329
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  • Anthony S. Fauci MD,

    Corresponding author
    1. NIAID, NIH, Bethesda, MD
    • American College of Rheumatology, 17 Executive Park Drive NE, Atlanta, GA 30329
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  • Randi Y. Leavitt MD, PHD,

    Corresponding author
    1. NIAID, NIH, Bethesda, MD
    • American College of Rheumatology, 17 Executive Park Drive NE, Atlanta, GA 30329
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  • Robert W. Lightfoot Jr Md,

    Corresponding author
    1. University of Kentucky, Lexington, KY
    • American College of Rheumatology, 17 Executive Park Drive NE, Atlanta, GA 30329
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  • Dennis J. McShane MD,

    Corresponding author
    1. Stanford University, Stanford, CA
    • American College of Rheumatology, 17 Executive Park Drive NE, Atlanta, GA 30329
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  • John A. Mills MD,

    Corresponding author
    1. Massachusetts General Hospital, Boston, MA
    • American College of Rheumatology, 17 Executive Park Drive NE, Atlanta, GA 30329
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  • Mary Betty Stevens MD,

    Corresponding author
    1. Johns Hopkins University, Baltimore, MD
    • American College of Rheumatology, 17 Executive Park Drive NE, Atlanta, GA 30329
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  • Stanley L. Wallace MD,

    Corresponding author
    1. SUNY Down-state Medical Center, Brooklyn, NY
    • American College of Rheumatology, 17 Executive Park Drive NE, Atlanta, GA 30329
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  • Nathan J. Zvaifler MD

    Corresponding author
    1. University of California, San Diego, San Diego, CA
    • American College of Rheumatology, 17 Executive Park Drive NE, Atlanta, GA 30329
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Abstract

Criteria for the classification of Churg-Strauss syndrome (CSS) were developed by comparing 20 patients who had this diagnosis with 787 control patients with other forms of vasculitis. For the traditional format classification, 6 criteria were selected: asthma, eosinophilia >10% on differential white blood cell count, mononeuropathy (including multiplex) or polyneuropathy, non-fixed pulmonary infiltrates on roentgenography, paranasal sinus abnormality, and biopsy containing a blood vessel with extravascular eosinophils. The presence of 4 or more of these 6 criteria yielded a sensitivity of 85% and a specificity of 99.7%. A classification tree was also constructed with 3 selected criteria: asthma, eosinophilia >10% on differential white blood cell count, and history of documented allergy other than asthma or drug sensitivity. If a subject has eosinophilia and a documented history of either asthma or allergy, then that subject is classified as having CSS. For the tree classification, the sensitivity was 95% and the specificity was 99.2%. Advantages of the traditional format compared with the classification tree format, when applied to patients with systemic vasculitis, and their comparison with earlier work on CSS are discussed.

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