The American College of Rheumatology 1990 criteria for the classification of henoch-schönlein purpura

Authors

  • 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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  • 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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  • 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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  • 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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  • 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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  • 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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  • 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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  • 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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  • 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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  • 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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  • 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 identifying Henoch-Schönlein Purpura (HSP) and distinguishing HSP from other forms of systemic arteritis were developed by comparing the manifestations in 85 patients who had HSP with those of 722 control patients with other forms of vasculitis. By the traditional format of choosing different combinations of candidate criteria and comparing the combinations for their ability to separate HSP cases from controls, 4 criteria were identified: age ≤20 years at disease onset, palpable purpura, acute abdominal pain, and biopsy showing granulocytes in the walls of small arterioles or venules. The presence of any 2 or more of these criteria distinguish HSP from other forms of vasculitis with a sensitivity of 87.1% and a specificity of 87.7%. The criteria selected by a classification tree method were similar: palpable purpura, age ≤20 years at disease onset, biopsy showing granulocytes around arterioles or venules, and gastrointestinal bleeding. These were able to distinguish HSP from other forms of vasculitis with a sensitivity of 89.4% and a specificity of 88.1%.

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