Title

2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative.

Authors

Frank van den Hoogen, St. Maartenskliniek and Radboud University Nijmegen Medical Centre
Dinesh Khanna, University of Michigan, Ann Arbor
Jaap Fransen, Radboud University Nijmegen Medical Centre
Sindhu R Johnson, Toronto Western Hospital, Mount Sinai Hospital, University of Toronto
Murray Baron, Jewish General Hospital and McGill University
Alan Tyndall, Felix Platter Spital and University of Basel
Marco Matucci-Cerinic, University of Florence
Raymond P Naden, Auckland City Hospital, New Zealand Health Ministry
Thomas A Medsger, University of Pittsburgh School of Medicine
Patricia E Carreira, Hospital Universitario
Gabriela Riemekasten, Charité University Medicine Berlin, German Rheumatology Research Center, Leibniz Institute
Philip J Clements, Department of Medicine, David Geffen School of Medicine at UCLA
Christopher P Denton, Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital
Oliver Distler, Department of Rheumatology, University Hospital Zurich
Yannick Allanore, Rheumatology A Department, Université Paris Descartes, Hôpital Cochin
Daniel E Furst, Department of Medicine, David Geffen School of Medicine at UCLA
Armando Gabrielli, Dipartimento di Scienze Cliniche e Molecolari- Clinica Medica Università Politecnica delle Marche Ancona, Italy
Maureen D Mayes, The University of Texas Health Science Center-Houston
Jacob M van Laar, Musculoskeletal Research Group, Institute of Cellular Medicine, The Medical School, Newcastle upon Tyne, United Kingdom
James R Seibold, Scleroderma Research Consultants, Avon, Connecticut, USA
Laszlo Czirjak, Department of Rheumatology and Immunology, Clinic Center, University of Pécs, Hungary
Virginia D Steen, Department of Medicine, Division of Rheumatology, Clinical Immunology and Allergy, Georgetown University School of Medicine
Murat Inanc, Istanbul University, Istanbul Medical Faculty, Department of Internal Medicine, Division of Rheumatology
Otylia Kowal-Bielecka, Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland
Ulf Müller-Ladner, Justus-Liebig University Giessen, Department of Rheumatology and Clinical Immunology, Kerckhoff Clinic
Gabriele Valentini, Rheumatology Unit, Second University of Naples, Naples, Italy
Douglas J Veale, St Vincent's University Hospital, Dublin, Ireland
Madelon C Vonk, Department of Rheumatology, Radboud University Nijmegen Medical Centre
Ulrich A Walker, Rheumatology Department, University of Basel
Lorinda Chung, Department of Medicine, Division of Immunology and Rheumatology, Stanford University
David H Collier, Division of Rheumatology, University of Colarado
Mary Ellen Csuka, Department of Rheumatology, Medical College Wisconsin, Milwaukee, USA
Barri J Fessler, Division of Clinical Immunology and Rheumatology, The University of Alabama at Birmingham
Serena Guiducci, Dept Biomedicine, Div Rheumatology AOUC, & Dept Medicine, Denothe Centre, Univ Florence, Italy
Ariane Herrick, University of Manchester, Manchester, UK, and Salford Royal National Health Service Foundation Trust
Vivien M Hsu, Robert Wood Johnson Medical School, Scleroderma Center, New Brunswick, New Jersey, USA
Sergio A. Jimenez, Jefferson Institute of Molecular Medicine, ConnectiveTissue Diseases Section, Scleroderma Center, Thomas Jefferson UniversityFollow
Bashar Kahaleh, Department of Medicine, Division of Rheumatology, University of Toledo
Peter A Merkel, Section of Rheumatology and the Clinical Epidemiology Unit, Boston University School of Medicine
Stanislav Sierakowski, Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland
Richard M Silver, Department of Medicine, Division of Rheumatology & Immunology, Medical University of South Carolina
Robert W Simms, Section of Rheumatology, Boston University School of Medicine
John Varga, Division of Rheumatology, Northwestern University Feinberg School of Medicine
Janet E Pope, Department of Medicine, Division of Rheumatology, St Joseph Health Care, University of Western Ontario

Document Type

Article

Publication Date

11-1-2013

Comments

This article has been peer reviewed. It was published in: Arthritis and Rheumatism.

Volume 65, Issue 11, November 2013, Pages 2737-2747.

The published version is available at DOI: 10.1002/art.38098

Copyright © 2013 by the American College of Rheumatology

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, 7 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.

CONCLUSION: 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.