Open Access
Open access
volume 72 issue 11 pages 1747-1755

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

Frank van den Hoogen 1
Dinesh Khanna 2
Jaap Fransen 3
Sindhu R. Johnson 4
Murray Baron 5
Alan Tyndall 6
Marco Matucci-Cerinic 7
Raymond P Naden 8
THOMAS A. MEDSGER 9
Patricia E. Carreira 10
Gabriela Riemekasten 11, 12
PHILIP J. CLEMENTS 13
Christopher P. Denton 14
Oliver Distler 15
Yannick Allanore 16
Daniel E Furst 13
Armando Gabrielli 17
Maureen D. Mayes 18
Jacob M. van Laar 19
James R. Seibold 20
Laszlo Czirjak 21
Virginia D. Steen 22
Murat Inanc 23
Otylia Kowal-Bielecka 24
Ulf Müller-Ladner 25
Gabriele Valentini 26
Douglas J. Veale 27
Madelon C Vonk 3
Ulrich A. Walker 6
Lorinda Chung 28
David H. Collier 29, 30
Mary Ellen Csuka 31
Barri J. Fessler 32
Serena Guiducci 7
Ariane Herrick 33, 34
Vivien M. Hsu 35
Sergio Jimenez 36
Bashar Kahaleh 37
Peter A. Merkel 38, 39
Stanislav Sierakowski 24
Richard M Silver 40
Robert W. Simms 39
John Varga 41
Janet E. Pope 42
12
 
German Rheumatology Research Center, and Leibniz Institute
20
 
Scleroderma Research Consultants
30
 
Amgen
34
 
Salford Royal National Health Service Foundation Trust
Publication typeJournal Article
Publication date2013-10-03
scimago Q1
wos Q1
SJR5.731
CiteScore33.2
Impact factor20.6
ISSN00034967, 14682060
General Biochemistry, Genetics and Molecular Biology
Immunology
Immunology and Allergy
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, 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, Raynaud9s 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.

Found 
Found 

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GOST |
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GOST Copy
van den Hoogen F. et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative // Annals of the Rheumatic Diseases. 2013. Vol. 72. No. 11. pp. 1747-1755.
GOST all authors (up to 50) Copy
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., Ellen Csuka M., 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., Pope J. E. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative // Annals of the Rheumatic Diseases. 2013. Vol. 72. No. 11. pp. 1747-1755.
RIS |
Cite this
RIS Copy
TY - JOUR
DO - 10.1136/annrheumdis-2013-204424
UR - https://doi.org/10.1136/annrheumdis-2013-204424
TI - 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative
T2 - Annals of the Rheumatic Diseases
AU - van den Hoogen, Frank
AU - Khanna, Dinesh
AU - Fransen, Jaap
AU - Johnson, Sindhu R.
AU - Baron, Murray
AU - Tyndall, Alan
AU - Matucci-Cerinic, Marco
AU - Naden, Raymond P
AU - MEDSGER, THOMAS A.
AU - Carreira, Patricia E.
AU - Riemekasten, Gabriela
AU - CLEMENTS, PHILIP J.
AU - Denton, Christopher P.
AU - Distler, Oliver
AU - Allanore, Yannick
AU - Furst, Daniel E
AU - Gabrielli, Armando
AU - Mayes, Maureen D.
AU - van Laar, Jacob M.
AU - Seibold, James R.
AU - Czirjak, Laszlo
AU - Steen, Virginia D.
AU - Inanc, Murat
AU - Kowal-Bielecka, Otylia
AU - Müller-Ladner, Ulf
AU - Valentini, Gabriele
AU - Veale, Douglas J.
AU - Vonk, Madelon C
AU - Walker, Ulrich A.
AU - Chung, Lorinda
AU - Collier, David H.
AU - Ellen Csuka, Mary
AU - Fessler, Barri J.
AU - Guiducci, Serena
AU - Herrick, Ariane
AU - Hsu, Vivien M.
AU - Jimenez, Sergio
AU - Kahaleh, Bashar
AU - Merkel, Peter A.
AU - Sierakowski, Stanislav
AU - Silver, Richard M
AU - Simms, Robert W.
AU - Varga, John
AU - Pope, Janet E.
PY - 2013
DA - 2013/10/03
PB - Elsevier
SP - 1747-1755
IS - 11
VL - 72
PMID - 24092682
SN - 0003-4967
SN - 1468-2060
ER -
BibTex |
Cite this
BibTex (up to 50 authors) Copy
@article{2013_van den Hoogen,
author = {Frank van den Hoogen and Dinesh Khanna and Jaap Fransen and Sindhu R. Johnson and Murray Baron and Alan Tyndall and Marco Matucci-Cerinic and Raymond P Naden and THOMAS A. MEDSGER and Patricia E. Carreira and Gabriela Riemekasten and PHILIP J. CLEMENTS and Christopher P. Denton and Oliver Distler and Yannick Allanore and Daniel E Furst and Armando Gabrielli and Maureen D. Mayes and Jacob M. van Laar and James R. Seibold and Laszlo Czirjak and Virginia D. Steen and Murat Inanc and Otylia Kowal-Bielecka and Ulf Müller-Ladner and Gabriele Valentini and Douglas J. Veale and Madelon C Vonk and Ulrich A. Walker and Lorinda Chung and David H. Collier and Mary Ellen Csuka and Barri J. Fessler and Serena Guiducci and Ariane Herrick and Vivien M. Hsu and Sergio Jimenez and Bashar Kahaleh and Peter A. Merkel and Stanislav Sierakowski and Richard M Silver and Robert W. Simms and John Varga and Janet E. Pope},
title = {2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative},
journal = {Annals of the Rheumatic Diseases},
year = {2013},
volume = {72},
publisher = {Elsevier},
month = {oct},
url = {https://doi.org/10.1136/annrheumdis-2013-204424},
number = {11},
pages = {1747--1755},
doi = {10.1136/annrheumdis-2013-204424}
}
MLA
Cite this
MLA Copy
van den Hoogen, Frank, et al. “2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative.” Annals of the Rheumatic Diseases, vol. 72, no. 11, Oct. 2013, pp. 1747-1755. https://doi.org/10.1136/annrheumdis-2013-204424.