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Ann Rheum Dis 72:1747-1755 doi:10.1136/annrheumdis-2013-204424
  • Criteria

2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative

  1. Janet E Pope41
  1. 1St. Maartenskliniek and Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
  2. 2University of Michigan, Ann Arbor, Michigan, USA
  3. 3Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
  4. 4Toronto Western Hospital, Mount Sinai Hospital, and University of Toronto, Toronto, Ontario, Canada
  5. 5Jewish General Hospital and McGill University, Montreal, Quebec, Canada
  6. 6Felix Platter Spital and University of Basel, Basel, Switzerland
  7. 7University of Florence, Florence, Italy
  8. 8Auckland City Hospital and New Zealand Health Ministry, Auckland, New Zealand
  9. 9University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
  10. 10Hospital Universitario 12 de Octubre, Madrid, Spain
  11. 11Charité University Medicine Berlin, German Rheumatology Research Center, and Leibniz Institute, Berlin, Germany
  12. 12 University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, USA
  13. 13Royal Free Hospital and University College London Medical School, London, UK
  14. 14University Hospital Zurich, Zurich, Switzerland
  15. 15Université Paris Descartes and Hôpital Cochin, AP-HP, Paris, France
  16. 16Clinica Medica Università Politecnica delle Marche, Ancona, Italy
  17. 17University of Texas Health Science Center, Houston, Texas, USA
  18. 18Newcastle University Medical School, Newcastle upon Tyne, UK
  19. 19Scleroderma Research Consultants, Avon, Connecticut, USA
  20. 20University of Pécs Medical Center, Pécs, Hungary
  21. 21Georgetown University School of Medicine, Washington, DC, USA
  22. 22Istanbul University, Istanbul, Turkey
  23. 23Medical University of Bialystok, Bialystok, Poland
  24. 24Justus-Liebig University Giessen and Kerckhoff Clinic, Bad Nauheim, Germany
  25. 25Second University of Naples, Naples, Italy
  26. 26St. Vincent's University Hospital, Dublin, Ireland
  27. 27Stanford University School of Medicine, Stanford, California, USA
  28. 28University of Colorado Medical School and Denver Health Medical Center, Denver, Colorado, USA
  29. 29Amgen, Thousand Oaks, California, USA
  30. 30 Medical College of Wisconsin, Milwaukee, Michigan, USA
  31. 31University of Alabama at Birmingham, Birmingham, UK
  32. 32University of Manchester, Manchester, UK
  33. 33Salford Royal National Health Service Foundation Trust, Salford, UK
  34. 34Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA
  35. 35Thomas Jefferson University, Philadelphia, Pennsylvania, USA
  36. 36University of Toledo, Toledo, Ohio, USA
  37. 37University of Pennsylvania, Philadelphia, Pennsylvania, USA
  38. 38Boston University School of Medicine, Boston, Massachusetts, USA
  39. 39 Medical University of South Carolina, Charleston, South Carolina, USA
  40. 40Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
  41. 41St. Joseph's Health Care London and University of Western Ontario, London, Ontario, Canada
  1. Correspondence to Dr Frank van den Hoogen, Department of Rheumatology, St. Maartenskliniek, PO Box 9011, Nijmegen 6500 GM, The Netherlands; F.vandenHoogen{at}maartenskliniek.nl or Janet E Pope, St. Joseph's Health Care London, 268 Grosvenor Street, London, ON, Canada N6A 4V2; janet.pope{at}sjhc.london.on.ca.
  • Accepted 9 August 2013

Abstract

Objective The 1980 American College of Rheumatology (ACR) classification criteria for systemic sclerosis (SSc) lack sensitivity for early SSc and limited cutaneous SSc. The present work, by a joint committee of the ACR and the European League Against Rheumatism (EULAR), was undertaken for the purpose of developing new classification criteria for SSc.

Methods Using consensus methods, 23 candidate items were arranged in a multicriteria additive point system with a threshold to classify cases as SSc. The classification system was reduced by clustering items and simplifying weights. The system was tested by (1) determining specificity and sensitivity in SSc cases and controls with scleroderma-like disorders, and (2) validating against the combined view of a group of experts on a set of cases with or without SSc.

Results It was determined that skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for the patient to be classified as having SSc; if that is not present, seven additive items apply, with varying weights for each: skin thickening of the fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, interstitial lung disease or pulmonary arterial hypertension, Raynaud's phenomenon, and SSc-related autoantibodies. Sensitivity and specificity in the validation sample were, respectively, 0.91 and 0.92 for the new classification criteria and 0.75 and 0.72 for the 1980 ACR classification criteria. All selected cases were classified in accordance with consensus-based expert opinion. All cases classified as SSc according to the 1980 ACR criteria were classified as SSc with the new criteria, and several additional cases were now considered to be SSc.

Conclusions The ACR/EULAR classification criteria for SSc performed better than the 1980 ACR criteria for SSc and should allow for more patients to be classified correctly as having the disease.

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