Dr. Khanna has received consulting fees from Bayer, Bristol-Myers Squibb, Digna Biotech, InterMune, Gilead, and Merck (less than $10,000 each) and consulting and speaking fees from Actelion and United Therapeutics (more than $10,000 each).
2013 Classification Criteria for Systemic Sclerosis: An American College of Rheumatology/European League Against Rheumatism Collaborative Initiative†
Article first published online: 3 OCT 2013
Copyright © 2013 by the American College of Rheumatology
Arthritis & Rheumatism
Volume 65, Issue 11, pages 2737–2747, November 2013
How to Cite
van den Hoogen, F., Khanna, D., Fransen, J., Johnson, S. R., Baron, M., Tyndall, A., Matucci-Cerinic, M., Naden, R. P., Medsger, T. A., Carreira, P. E., Riemekasten, G., Clements, P. J., Denton, C. P., Distler, O., Allanore, Y., Furst, D. E., Gabrielli, A., Mayes, M. D., van Laar, J. M., Seibold, J. R., Czirjak, L., Steen, V. D., Inanc, M., Kowal-Bielecka, O., Müller-Ladner, U., Valentini, G., Veale, D. J., Vonk, M. C., Walker, U. A., Chung, L., Collier, D. H., Csuka, M. E., Fessler, B. J., Guiducci, S., Herrick, A., Hsu, V. M., Jimenez, S., Kahaleh, B., Merkel, P. A., Sierakowski, S., Silver, R. M., Simms, R. W., Varga, J. and Pope, J. E. (2013), 2013 Classification Criteria for Systemic Sclerosis: An American College of Rheumatology/European League Against Rheumatism Collaborative Initiative. Arthritis & Rheumatism, 65: 2737–2747. doi: 10.1002/art.38098
This article is published simultaneously in the November 2013 issue of Annals of the Rheumatic Diseases.
- Issue published online: 28 OCT 2013
- Article first published online: 3 OCT 2013
- Manuscript Accepted: 16 JUL 2013
- Manuscript Received: 26 JAN 2013
- American College of Rheumatology
- European League Against Rheumatism
- Scleroderma Foundation (New Investigator award)
- NIH (National Institute of Arthritis and Musculoskeletal and Skin Diseases). Grant Number: K24-AR-063120
- Canadian Institutes of Health Research (Clinician Scientist award)
- Norton-Evans Fund for Scleroderma Research
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.
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.
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.
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.