2013 classification criteria for systemic sclerosis

An American college of rheumatology/European league against rheumatism collaborative initiative

Frank Van Den Hoogen, Dinesh Khanna, Jaap Fransen, Sindhu R. Johnson, Murray Baron, Alan Tyndall, Marco Matucci-Cerinic, Raymond P. Naden, Thomas A. Medsger, Patricia E. Carreira, Gabriela Riemekasten, Philip J. Clements, Christopher P. Denton, Oliver Distler, Yannick Allanore, Daniel E. Furst, Armando Gabrielli, Maureen D. Mayes, Jacob M. Van Laar, James R. Seibold & 24 others L. Czirják, Virginia D. Steen, Murat Inanc, Otylia Kowal-Bielecka, Ulf Müller-Ladner, Gabriele Valentini, Douglas J. Veale, Madelon C. Vonk, Ulrich A. Walker, Lorinda Chung, David H. Collier, Mary Ellen Csuka, Barri J. Fessler, Serena Guiducci, Ariane Herrick, Vivien M. Hsu, Sergio Jimenez, Bashar Kahaleh, Peter A. Merkel, Stanislav Sierakowski, Richard M. Silver, Robert W. Simms, John Varga, Janet E. Pope

Research output: Contribution to journalArticle

585 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1747-1755
Number of pages9
JournalAnnals of the Rheumatic Diseases
Volume72
Issue number11
DOIs
Publication statusPublished - Nov 2013

Fingerprint

Systemic Scleroderma
Rheumatology
Rheumatic Diseases
Skin
Pulmonary diseases
Autoantibodies
Fingers
Metacarpophalangeal Joint
Weights and Measures
Sensitivity and Specificity
Telangiectasis
Raynaud Disease
Interstitial Lung Diseases
Expert Testimony
Pulmonary Hypertension
Cluster Analysis

ASJC Scopus subject areas

  • Rheumatology
  • Immunology
  • Biochemistry, Genetics and Molecular Biology(all)
  • Immunology and Allergy

Cite this

2013 classification criteria for systemic sclerosis : An American college of rheumatology/European league against rheumatism collaborative initiative. / Van Den Hoogen, Frank; Khanna, Dinesh; Fransen, Jaap; Johnson, Sindhu R.; Baron, Murray; Tyndall, Alan; Matucci-Cerinic, Marco; Naden, Raymond P.; Medsger, Thomas A.; Carreira, Patricia E.; Riemekasten, Gabriela; Clements, Philip J.; Denton, Christopher P.; Distler, Oliver; Allanore, Yannick; Furst, Daniel E.; Gabrielli, Armando; Mayes, Maureen D.; Van Laar, Jacob M.; Seibold, James R.; Czirják, L.; Steen, Virginia D.; Inanc, Murat; Kowal-Bielecka, Otylia; Müller-Ladner, Ulf; Valentini, Gabriele; Veale, Douglas J.; Vonk, Madelon C.; Walker, Ulrich A.; Chung, Lorinda; Collier, David H.; Csuka, Mary Ellen; Fessler, Barri J.; Guiducci, Serena; Herrick, Ariane; Hsu, Vivien M.; Jimenez, Sergio; Kahaleh, Bashar; Merkel, Peter A.; Sierakowski, Stanislav; Silver, Richard M.; Simms, Robert W.; Varga, John; Pope, Janet E.

In: Annals of the Rheumatic Diseases, Vol. 72, No. 11, 11.2013, p. 1747-1755.

Research output: Contribution to journalArticle

Van Den Hoogen, F, Khanna, D, Fransen, J, Johnson, SR, Baron, M, Tyndall, A, Matucci-Cerinic, M, Naden, RP, Medsger, TA, Carreira, PE, Riemekasten, G, Clements, PJ, Denton, CP, Distler, O, Allanore, Y, Furst, DE, Gabrielli, A, Mayes, MD, Van Laar, JM, Seibold, JR, Czirják, L, Steen, VD, Inanc, M, Kowal-Bielecka, O, Müller-Ladner, U, Valentini, G, Veale, DJ, Vonk, MC, Walker, UA, Chung, L, Collier, DH, Csuka, ME, Fessler, BJ, Guiducci, S, Herrick, A, Hsu, VM, Jimenez, S, Kahaleh, B, Merkel, PA, Sierakowski, S, Silver, RM, Simms, RW, Varga, J & Pope, JE 2013, '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, pp. 1747-1755. https://doi.org/10.1136/annrheumdis-2013-204424
Van Den Hoogen, Frank ; Khanna, Dinesh ; Fransen, Jaap ; Johnson, Sindhu R. ; Baron, Murray ; Tyndall, Alan ; Matucci-Cerinic, Marco ; Naden, Raymond P. ; Medsger, Thomas A. ; Carreira, Patricia E. ; Riemekasten, Gabriela ; Clements, Philip J. ; Denton, Christopher P. ; Distler, Oliver ; Allanore, Yannick ; Furst, Daniel E. ; Gabrielli, Armando ; Mayes, Maureen D. ; Van Laar, Jacob M. ; Seibold, James R. ; Czirják, L. ; Steen, Virginia D. ; Inanc, Murat ; Kowal-Bielecka, Otylia ; Müller-Ladner, Ulf ; Valentini, Gabriele ; Veale, Douglas J. ; Vonk, Madelon C. ; Walker, Ulrich A. ; Chung, Lorinda ; Collier, David H. ; Csuka, Mary Ellen ; Fessler, Barri J. ; Guiducci, Serena ; Herrick, Ariane ; Hsu, Vivien M. ; Jimenez, Sergio ; Kahaleh, Bashar ; Merkel, Peter A. ; Sierakowski, Stanislav ; Silver, Richard M. ; Simms, Robert W. ; Varga, John ; Pope, Janet E. / 2013 classification criteria for systemic sclerosis : An American college of rheumatology/European league against rheumatism collaborative initiative. In: Annals of the Rheumatic Diseases. 2013 ; Vol. 72, No. 11. pp. 1747-1755.
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title = "2013 classification criteria for systemic sclerosis: An American college of rheumatology/European league against rheumatism collaborative initiative",
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.",
author = "{Van Den Hoogen}, Frank and Dinesh Khanna and Jaap Fransen and Johnson, {Sindhu R.} and Murray Baron and Alan Tyndall and Marco Matucci-Cerinic and Naden, {Raymond P.} and Medsger, {Thomas A.} and Carreira, {Patricia E.} and Gabriela Riemekasten and Clements, {Philip J.} and Denton, {Christopher P.} and Oliver Distler and Yannick Allanore and Furst, {Daniel E.} and Armando Gabrielli and Mayes, {Maureen D.} and {Van Laar}, {Jacob M.} and Seibold, {James R.} and L. Czirj{\'a}k and Steen, {Virginia D.} and Murat Inanc and Otylia Kowal-Bielecka and Ulf M{\"u}ller-Ladner and Gabriele Valentini and Veale, {Douglas J.} and Vonk, {Madelon C.} and Walker, {Ulrich A.} and Lorinda Chung and Collier, {David H.} and Csuka, {Mary Ellen} and Fessler, {Barri J.} and Serena Guiducci and Ariane Herrick and Hsu, {Vivien M.} and Sergio Jimenez and Bashar Kahaleh and Merkel, {Peter A.} and Stanislav Sierakowski and Silver, {Richard M.} and Simms, {Robert W.} and John Varga and Pope, {Janet E.}",
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TY - JOUR

T1 - 2013 classification criteria for systemic sclerosis

T2 - An American college of rheumatology/European league against rheumatism collaborative initiative

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 - Czirják, L.

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 - Csuka, Mary Ellen

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 - 2013/11

Y1 - 2013/11

N2 - 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.

AB - 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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