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

  1. Alfonse T. Masi MD, DRPH1,*,
  2. Gene G. Hunder MD Chair2,*,
  3. J. T. Lie MD3,*,
  4. Beat A. Michel MD4,*,
  5. Daniel A. Bloch PhD5,*,
  6. William P. Arend MD6,*,
  7. Leonard H. Calabrese DO7,*,
  8. Steven M. Edworthy MD8,*,
  9. Anthony S. Fauci MD9,*,
  10. Randi Y. Leavitt MD, PHD10,*,
  11. Robert W. Lightfoot Jr Md11,*,
  12. Dennis J. McShane MD12,*,
  13. John A. Mills MD13,*,
  14. Mary Betty Stevens MD14,*,
  15. Stanley L. Wallace MD15,*,
  16. Nathan J. Zvaifler MD16,*

Article first published online: 17 AUG 2010

DOI: 10.1002/art.1780330806

Issue

Arthritis & Rheumatism

Arthritis & Rheumatism

Volume 33, Issue 8, pages 1094–1100, August 1990

Additional Information

How to Cite

Masi, A. T., Hunder, G. G., Lie, J. T., Michel, B. A., Bloch, D. A., Arend, W. P., Calabrese, L. H., Edworthy, S. M., Fauci, A. S., Leavitt, R. Y., Lightfoot, R. W., McShane, D. J., Mills, J. A., Stevens, M. B., Wallace, S. L. and Zvaifler, N. J. (1990), The American College of Rheumatology 1990 criteria for the classification of churg-strauss syndrome (allergic granulomatosis and angiitis). Arthritis & Rheumatism, 33: 1094–1100. doi: 10.1002/art.1780330806

Author Information

  1. 1

    University of Illinois College of Medicine, Peoria, IL

  2. 2

    Mayo Clinic, Rochester, MN Subcommittee on Classification of Vasculitis

  3. 3

    Mayo Clinic, Rochester, MN

  4. 4

    Rheumaklinik Universitätsspital, Zurich, Switzerland

  5. 5

    Stanford University, Stanford, CA

  6. 6

    University of Colorado Health Science Center, Denver, CO

  7. 7

    Cleveland Clinic Foundation, Cleveland, OH

  8. 8

    University of Calgary, Calgary, Alberta, Canada

  9. 9

    NIAID, NIH, Bethesda, MD

  10. 10

    NIAID, NIH, Bethesda, MD

  11. 11

    University of Kentucky, Lexington, KY

  12. 12

    Stanford University, Stanford, CA

  13. 13

    Massachusetts General Hospital, Boston, MA

  14. 14

    Johns Hopkins University, Baltimore, MD

  15. 15

    SUNY Down-state Medical Center, Brooklyn, NY

  16. 16

    University of California, San Diego, San Diego, CA

*American College of Rheumatology, 17 Executive Park Drive NE, Atlanta, GA 30329

Publication History

  1. Issue published online: 17 AUG 2010
  2. Article first published online: 17 AUG 2010
  3. Manuscript Accepted: 3 APR 1990

Funded by

  • NIH. Grant Number: AM-21393

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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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