Brief Report

Arthritis Care & Research DOI 10.1002/acr.22451

The 2013 ACR/EULAR Classification Criteria for Systemic Sclerosis Out-perform the 1980 Criteria. Data from the Canadian Scleroderma Research Group Hebah Alhajeri1,2, Marie Hudson1,2,3, Marvin Fritzler4, Janet Pope5, Solène Tatibouet3, Janet Markland†6, David Robinson7, Niall Jones8, Nader Khalidi9,10, Peter Docherty11, Elzbieta Kaminska4, Ariel Masetto12, Evelyn Sutton13, Jean-Pierre Mathieu14, Sophie Ligier14, Tamara Grodzicky15, Sharon LeClercq4, Carter Thorne16, Geneviève Gyger1,2, Douglas Smith17, Paul R. Fortin18, Maggie Larché9,10, Murray Baron1,2 †

Deceased

Author institutional affiliations: 1Department of Medicine, McGill University, 845 Sherbrooke St W, Montreal, H3A 0G4, Quebec, Canada; 2Division of Rheumatology and 3

Lady Davis Institute, Jewish General Hospital, 3755 Côte Ste-Catherine, Montréal, H3T

1E3, Quebec, Canada; 4Faculty of Medicine, University of Calgary, 3330 Hospital Drive, Calgary, T2N 4N1, Alberta, Canada; 5Deparment of Medicine, University of Western Ontario, 1151 Richmond St, London, N6A 3K7, Ontario, Canada; 6Division of Rheumatology, University of Saskatchewan, 105Administration Pl, Saskatoon, S7N 5A1, Saskatchewan, Canada; 7Faculty of Medicine, University of Manitoba, 66 Chancellors Cir, Winnipeg, R3T 2N2, Manitoba, Canada; 8Department of Medicine, University of Alberta, 116 St and 85 Ave, Edmonton, T6G 2R3, Alberta, Canada; 9Department of Medicine, McMaster University, 1280 Main St W, Hamilton, L8S 4L8, Ontario, Canada; 10

Division of Rheumatology, St. Joseph’s Healthcare Hamilton, 50 Charlton Ave E,

Hamilton, L8N 4A6, Ontario, Canada; 11Division of Rheumatology, The Moncton Hospital, 135 Macbeath Ave, Moncton, E1C 6Z8, New Brunswick, Canada; 12 Department of Medicine, Université de Sherbooke, 2500 boul. de l’Université, This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process which may lead to differences between this version and the Version of Record. Please cite this article as an ‘Accepted Article’, doi: 10.1002/acr.22451 © 2014 American College of Rheumatology Received: Jan 13, 2014; Revised: May 20, 2014; Accepted: Aug 12, 2014

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Sherbrooke, J1K 2R1, Quebec, Canada; 13Division of Rheumatology, Department of Medicine, Dalhousie University, Halifax, B3H 4R2, Nova Scotia, Canada; 14Division of Rheumatology, Hôpital Maisonneuve-Rosemont, 5415 boul. de l’Assomption, Montreal, H1T 2M4, Quebec, Canada; 15Department of Rheumatology, Hôpital Notre-Dame, 1560 rue Sherbrooke E, Montreal, H2L 4M1, Quebec, Canada; 16Medicine, Southlake Regional Health Centre, 596 Davis Dr, Newmarket, L3Y 2P9, Ontario, Canada; 17

Division of Rheumatology, Faculty of Medicine, University of Ottawa, 75 Laurier Ave

E, Ottawa, K1N 6N5, Ontario, Canada; 18Faculty of Medicine, Université Laval, 2325 rue de l’Université, G1V 0A6, Quebec, Canada Academic degrees of authors: Hebah Alhajeri MD; Marie Hudson MD MPH; Marvin J. Fritzler PhD MD; Janet Pope MD; Solène Tatibouet MSc; Janet Markland MD MPH; David Robinson MD MSc; Niall Jones MD; Nader Khalidi MD; Peter Docherty MD; Elzbieta Kaminska MD; Ariel Masetto MD; Evelyn Sutton MD; Jean-Pierre Mathieu MD; Sophie Ligier MD; Tamara Grodzicky MD; Sharon LeClercq MD; Carter Thorne MD; Geneviève Gyger MD; Douglas Smith MD; Paul R. Fortin MD MPH; Maggie Larché MD PhD; Murray Baron MD Correspondence and request for reprints: Dr Murray Baron, Jewish General Hospital, Room A-725, 3755 Côte Sainte-Catherine Road, Montreal, Quebec, H3T 1E2, tel. 514340-8231, fax: 514-340-7906, e-mail: [email protected] Funding: The CSRG received funds from the Canadian Institutes of Health Research (CIHR) (grant #FRN 83518), the Scleroderma Society of Canada and its provincial Chapters, Scleroderma Society of Ontario, Sclérodermie Québec, Cure Scleroderma Foundation, INOVA Diagnostics Inc. (San Diego, CA), Dr. Fooke Laboratorien GmbH 2 John Wiley & Sons, Inc.

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(Neuss, Germany), Euroimmun (Lubeck, Germany), Mikrogen GmbH (Neuried, Germany), Fonds de la recherche en santé du Québec (FRSQ), the Canadian Arthritis Network (CAN), and the Lady Davis Institute of Medical Research of the Jewish General Hospital, Montreal, QC. The CSRG has also received educational grants from Pfizer and Actelion pharmaceuticals. Dr. Hudson is funded by the Fonds de la recherche en Santé du Québec. The funding sources had no role in the design of the study, analysis of the data, preparation of the manuscript and decision to submit for publication. Running title: 2013 ACR/EULAR SSc Criteria in the Canadian Scleroderma Research Group Cohort Key indexing terms: Systemic sclerosis (SSc), Classification criteria, Sine scleroderma. Word count: 2313

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ABSTRACT Objective: The goal of this study was to determine the sensitivity of the new 2013 classification criteria for systemic sclerosis (SSc) in an independent cohort of SSc subjects and to assess the contribution of individual items of the criteria to the overall sensitivity. Method: SSc subjects from the Canadian Scleroderma Research Group cohort were assessed. Sensitivity was determined in several subgroups of patients. In patients without the criterion of skin thickening proximal to the metacarpophalangeal joints (MCPs), we re-calculated sensitivity after removing individual criterion. Results: A total of 724 SSc patients were included. Most were females (86%), mean age was 55.8 years, mean disease duration was 10.9 years, and 59% had lcSSc. Overall, the sensitivity of the 2013 criteria was 98.3% compared to 88.3% for the 1980 criteria. This pattern was consistent among those with lcSSc (98.8% versus 85.6%), anti-centromere antibodies (98.9% vs 79.8%), disease duration ≤ 3 years (98.7% vs 84.7%) and no skin involvement proximal to the MCPs (97% vs 60%). In the latter sub-group, removing Raynaud’s phenomenon and sclerodactyly from the criteria reduced the sensitivity to 77% and 79%, respectively. Removing both sclerodactyly and puffy fingers reduced the sensitivity to 62%. Conclusion: The 2013 SSc classification criteria classify more SSc patients than the 1980 criteria. The improvement in sensitivity is most striking in those with lcSSc, especially those without skin involvement proximal to the MCPs. The addition of Raynaud’s

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phenomenon and puffy fingers to the 2013 criteria accounts for important gains in sensitivity.

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SIGNIFICANCE AND INNOVATIONS •

The 2013 ACR/EULAR Classification Criteria for SSc have marked increased sensitivity compared to the 1980 criteria.



The improved sensitivity is especially noted in those with lcSSc, especially with no skin involvement proximal to MCPs.



74% of subjects with scleroderma sine scleroderma are captured by the 2013 criteria.



The inclusion of Raynaud’s phenomenon and puffy fingers in the 2013 criteria accounts for important gains in sensitivity.

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INTRODUCTION Systemic sclerosis (SSc) is an autoimmune disease characterised by vasculopathy, immune dysregulation, and fibroblast dysfunction, causing tissue ischemia, autoantibody production and increased deposition of extracellular matrix(1). It is a heterogeneous disease, but 2 common subsets based on the extent of skin involvement are typically recognized, namely limited cutaneous SSc (lcSSC) with skin involvement distal to the elbows and knees, and diffuse cutaneous SSc (dcSSc) with skin involvement extending to the proximal limbs and/or trunk. The first widely accepted classification criteria for SSc were published as “preliminary” criteria in 1980 and were intentionally designed to be more specific than sensitive to minimize false positive diagnoses(2). However, the loss of sensitivity contributed to the exclusion of a considerable number of SSc subjects, particularly those with early and lcSSc disease. Since the publication of these criteria, the widespread clinical use of nailfold capillaroscopy and SSc specific autoantibodies, have facilitated the recognition of SSc, particularly in early and limited disease(3-6). Recently, a new set of criteria published by a joint committee of the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR), referred to here as the 2013 ACR/EULAR Classification Criteria for SSc, was developed primarily to increase the sensitivity of the old criteria while preserving specificity(7, 8). These new criteria include, among other variables, nailfold capillaroscopy and SSc-specific antibodies. In the development phase, the sensitivity and specificity of the new criteria were reported to be 95% and 93%, respectively.

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The objective of our study was to validate the sensitivity of the new criteria in an independent cohort of SSc subjects and in selected socio-demographic and clinical subgroups. We also sought to determine how individual items (eg. nailfold capillaroscopy and autoantibodies) contributed to the overall sensitivity of the new criteria.

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METHODS Study Design and Patients This was a cross-sectional study of a cohort of SSc patients. The study subjects consisted of those enrolled in the Canadian Scleroderma Research Group Registry (CSRG). Patients in this registry were recruited from 15 centers across Canada. They had a diagnosis of SSc made by an experienced rheumatologist, were aged >18 years, and fluent in English or French. They were not required to meet any formal classification criteria. The patients available for this study were those whose baseline visit was between September 2004 and July 2013. Only those CSRG patients who had a complete serological data (anti centromere, anti topoisomerase, and anti RNAIII) were included in the study. During the development phase of the ACR/EULAR, data from 127 CSRG subjects were used by the ACR/EULAR committee and these patients were excluded from this study. Ethics committee approval for the CSRG data collection protocol was obtained at McGill University (Montreal, Canada) and at all participating study sites. All subjects provided informed written consent to participate in the data collection protocol. SSc classification criteria The 1980 ACR preliminary classification criteria for SSc included skin thickening proximal to the metacarpophalangeal (MCP) joints as the major criterion, and sclerodactyly, digital pitting scars, and bibasilar pulmonary fibrosis as the minor criteria. A patient with either the major criteria or 2 out of 3 minor criteria was said to fulfil those criteria(2).

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The 2013 ACR/EULAR Classification Criteria for SSc employ a point system such that a score > 9 is needed to classify a patient with SSc(7, 8). The new criteria, similar to the preliminary ones, identified skin thickening proximal to MCPs as sufficient to classify a subject with SSc (9 points). Alternatively, 7 items with varying weights, namely skin thickening of the fingers (puffy fingers 2 points or sclerodactyly 4 points, counting the higher score only), fingertip lesions (digital tip ulcers 2 points or pitting scars 3 points, counting the higher score only), telangiectasia (2 points), abnormal nailfold capillaries (2 points), interstitial lung disease or pulmonary arterial hypertension (2 points), Raynaud’s phenomenon (3 points ) and SSc-specific autoantibodies (3 points) were included. Study variables Patients in the study cohort underwent a detailed standardized clinical exam. Demographic data (age, sex, ethnicity) was collected by patient self-report. Disease duration was determined from the onset of the first non-Raynaud’s disease manifestation. Patients were classified into limited and diffuse cutaneous subsets based on the definition of Leroy et al(9). Those with a clinical diagnosis of SSc but no skin involvement were classified as having scleroderma sine scleroderma. Raynaud’s phenomenon, puffy fingers, telangiectasias and digital pits were reported as binary variables (present or absent) by physician report. Abnormal nailfold capillaries (drop-outs, enlarged or giant capillaries) were identified by study physicians using a Dermlite© dermatoscope(10). The presence of interstitial lung disease (ILD) or pulmonary arterial hypertension (PAH) was determined according to the definitions of the 2013 ACR/EULAR Classification

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Criteria paper(7, 8). Serum was collected on all patients and sent to a central laboratory, Mitogen Advanced Diagnostics Laboratory, University of Calgary. Aliquots of sera are stored at -70 C until needed. Anti-centromere antibodies were assessed by indirect immunofluorescence on HEp-2000 substrate (ImmunoConcepts Inc., Sacramento, CA, USA), anti-RNA polymerase III (anti-RNA pol III ) antibodies were detected by ELISA (INOVA Diagnostics)(11) and anti-topoisomerase I antibodies (ATA) were assayed by an addressable laser bead immunoassay (ALBIA) using a commercially available kit (QUANTAPlex ENA 8, INOVA Diagnostics Inc., San Diego, CA) in a Luminex 100 (Luminex Corp., Austin, TX), platform according to protocols previously described(11). Statistical analysis Descriptive statistics were used to summarize the baseline characteristics of the patients. The sensitivity of 2013 ACR/EULAR and the 1980 ACR classification criteria was calculated. McNemar tests were performed to compare the sensitivities of the 2013 and 1980 criteria. In patients who did not have the 2013 major criterion of skin thickening proximal to the metacarpophalangeal joints, we tested the importance of the minor criteria by re-calculating sensitivity after removing individual criterion, alone or in combination. Missing data were not imputed. When data were missing, proportions were calculated using the available denominator. P-values less than 0.05 were considered as significant. All statistical analyses were performed with SAS v.9.2 (SAS Institute, USA).

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RESULTS This study included 724 SSc subjects (Table 1). Data were available for all variables in over 95% of subjects except for PAH where 8% had missing data. The majority of subjects were female (86%), mean age was 55.8 (+12.2) yrs and mean disease duration was 10.9 (+9) years. Of these, 59% had lcSSc and 38% dcSSc, 4% had scleroderma sine scleroderma and 76% had skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints. Puffy fingers were present in 63% of study subjects, sclerodactyly in 98%, digital tip ulcers in 54%, digital pitting scars in 43%, telangiectasia in 76%, abnormal nailfold capillaries in 76%, pulmonary arterial hypertension in 5%, interstitial lung disease in 33%, and Raynaud’s phenomenon in 98%. SSc-specific autoantibodies were present in two thirds of subjects: 36% had anticentromere, 15% anti-topoisomerase and 17% anti-RNA pol III antibodies. The overall sensitivity of the 2013 classification criteria was 98.3% compared to 88.3% for the 1980 criteria (P < 0.001; Table 2). The sensitivities of the 2013 and 1980 criteria were 100% in patients with diffuse cutaneous disease. However, in patients with limited disease, the sensitivities of the 2013 and 1980 criteria were 98.8% and 85.6%, respectively (P < 0.001) and in those with scleroderma sine scleroderma, the sensitivities of the 2013 and 1980 criteria were 74.1% and 11.1%, respectively (P < 0.001). Among those with disease duration < 3 years, the sensitivities of the 2013 and 1980 criteria were 98.7% and 84.7%, respectively (P < 0.001). Among the 262 patients with anti-centromere antibodies, the sensitivities of the 2013 and 1980 criteria were 98.9% and 79.8%, respectively (P < 0.001). Finally, among patients with both anti-centromere antibodies

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and disease duration of < 3 years, the sensitivities of 2013 and 1980 criteria were 97.7% and 63.6%, respectively (P < 0.001). The impact of the variables included in the 2013 ACR/EULAR classification criteria. We sought to determine how individual items included in the 2013 criteria contributed to the increased overall sensitivity (Table 3). Specifically, we analyzed those patients who did not meet the sufficient criteria of both the 2013 and the 1980 criteria, i.e. skin thickening proximal to the MCP joints. Among those who were classified as lcSSc who did not have skin thickening proximal to the MCP joints (n=150), the items that had the largest impact on sensitivity were sclerodactyly, the combination of puffy fingers and sclerodactyly, Raynaud’s phenomenon, and the combination of abnormal nailfold capillaries and anti-centromere antibodies, since removing these items decreased sensitivity from 97% to 79%, 62%, 77%, and 85%, respectively. Among those who did not have skin thickening proximal to the metacarpophalangeal joints and who were classified with scleroderma sine scleroderma, the sensitivity of the new classification criteria (74%) dropped dramatically when the following items were removed: telangiectasia (30%), abnormal nailfold capillaries (33%) and Raynaud’s phenomenon (26%), anti-centromere antibody (52%) and the combination of abnormal nailfold capillaries and anti-centromere antibodies (22%). A total of 8% of subjects were missing data regarding PAH status according to right heart catheterization, so we checked to determine if the missing data affected our observations. The only patient with missing PAH data who did not meet the new ACR criteria also had ILD which means that the score for that criteria item would have been the same no matter

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what the PAH status. All other cases with missing PAH data met the 2013 criteria based on other criteria so removing PAH would not have reduced the sensitivity of the criteria. Patients who did not meet the 2013 classification criteria (Table 4): Twelve patients did not meet the new classification criteria: 5 with lcSSc, and 7 with scleroderma sine scleroderma. All of them were females and all but one had Raynaud’s phenomenon. Nine of them had 3 items from the 2013 criteria, most commonly Raynaud’s, abnormal nailfold capillaries and telangiectasia. A fourth criterion item such as ACA would have permitted classification as SSc.

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DISCUSSION In this study, we confirmed that the 2013 classification criteria for SSc were highly sensitive (98.3%), and that this sensitivity was significantly greater than that of the 1980 criteria (88.3%). The improvement in sensitivity is most striking in lcSSc, defined either by cutaneous examination or by positive ACA and especially in those with recent onset ACA positive disease. There was also a marked improvement in sensitivity in the scleroderma sine scleroderma group from the 1980 (11.1%) to 2013 criteria (74.1%). Although this group is relatively rare, it was essentially totally missed by the old criteria. In those lcSSc subjects that do not meet the single criteria of proximal skin thickening, puffy fingers and sclerodactyly together had a large impact on the sensitivity of the new classification criteria as did the inclusion of Raynaud’s phenomenon in the new criteria. Indeed, considering that sclerodactyly was in the old criteria, the inclusion of Raynaud’s phenomenon in the new criteria appears to be the single most important innovation in increasing sensitivity. Several other items, particularly telangiectasia, abnormal nailfold capillaries and anticentromere antibodies also contributed slightly to the improvement in the sensitivity of the 2013 criteria. Puffy fingers was also found to be an important item in preliminary criteria for very early diagnosis of systemic sclerosis that were identified by the EULAR Scleroderma Trials and Research Group (EUSTAR)(12). Similarly, in the sine subset, the impact of the items that were not included in the old classification criteria was very pronounced, especially for Raynaud’s phenomenon, telangiectasia, abnormal nailfold capillaries, and SSc related antibodies. Subjects who did not meet the new classification criteria often had 3 features of SSc and thus a clinical picture that could be considered somewhat controversial in terms of being 15 John Wiley & Sons, Inc.

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labeled as SSc. For example, one such phenotype would be sclerodactyly, telangiectasia and abnormal capillaries but no specific antibodies. One might conclude that the ACR/EULAR committee adopted a conservative approach by not classifying such patients as SSc and, because these cases could be considered controversial, this approach seems justified. As inclusion of a patient in our cohort was at the discretion of the participating rheumatologist, the fact that there were so few such cases perhaps reflects the uncertainty about designating these patients as SSc. This study is not without limitations. The patients in the CSRG Registry are generally recruited as outpatients and have a long mean disease duration of over 10 years. Thus, a survivor bias cannot be excluded. Nevertheless, the subjects covered the full spectrum of disease, including a fair proportion with early disease (21%). Hence, our findings are generalizable within the context of the study sampling frame. Eight percent of subjects were missing data regarding PAH status according to right heart catheterization. We found that this missing data had no effect on classification. Indeed, the frequency of PAH in this study was relatively low (5%) suggesting that we may have under-ascertained the presence of PAH. If PAH were important for the 2013 criteria, such misclassification bias would, if anything, have resulted in a more conservative estimate of sensitivity. Therefore PAH does not appear to be a particularly important element in the criteria. Another limitation was that we did not have a comparison group and therefore we were unable to calculate the specificity of the 2013 ACR/EULAR classification criteria. The strength of the current study lies in its large sample size. SSc is a rare and heterogeneous disease. Collaborative research and large, detailed data sets are necessary to pursue meaningful research in this disease(13). 16 John Wiley & Sons, Inc.

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Our analysis demonstrated the high sensitivity of the 2013 classification criteria in a large cohort of SSc patients, as well as in the subsets of patients with limited and/or early disease. Raynaud’s phenomenon was the single most important new item that contributed to this high sensitivity, and secondarily, sclerodactyly, puffy fingers, abnormal nailfold capillaries and anti-centromere antibodies, alone or in combination, also contributed to the improved sensitivity of the 2013 criteria. Further confirmation of the specificity of the new criteria will require additional studies in comparison mimicking diseases.

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REFERENCES 1. Wollheim F. Classification of systemic sclerosis. Visions and reality. Rheumatology 2005;44(10):1212-1216. 2. Masi AT, Subcommittee For Scleroderma Criteria of the American Rheumatism Association D, Therapeutic Criteria C. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis & Rheumatism 1980;23(5):581-590. 3. LeRoy EC, Medsger TA. Criteria for the classification of early systemic sclerosis. The Journal of Rheumatology 2001;28(7):1573-1576. 4. Hudson M, Taillefer S, Steele R, Dunne J, Johnson SR, Jones N, et al. Improving the sensitivity of the American College of Rheumatology classification criteria for systemic sclerosis. Clinical and experimental rheumatology 2007;25(5):754-7. 5. Lonzetti LS, Joyal F, Raynauld JP, Roussin A, Goulet JR, Rich E, et al. Updating the American College of Rheumatology preliminary classification criteria for systemic sclerosis: addition of severe nailfold capillaroscopy abnormalities markedly increases the sensitivity for limited scleroderma. Arthritis and rheumatism 2001;44(3):735-6. 6. LeRoy EC, Medsger TA, Jr. Criteria for the classification of early systemic sclerosis. The Journal of Rheumatology 2001;28(7):1573-6. 7. den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Arthritis & Rheumatism 2013. 8. van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, 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;72(11):1747-55. 9. LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA, Jr., et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988;15(2):202-5. 10. Baron M, Bell M, Bookman A, Buchignani M, Dunne J, Hudson M, et al. Office capillaroscopy in systemic sclerosis. Clin Rheumatol 2007;26(8):1268-74. 11. Santiago M, Baron M, Hudson M, Burlingame RW, Fritzler MJ. Antibodies to RNA polymerase III in systemic sclerosis detected by ELISA. The Journal of Rheumatology 2007;34(7):1528-1534. 12. Minier T, Guiducci S, Bellando-Randone S, Bruni C, Lepri G, Czirják L, et al. Preliminary analysis of the Very Early Diagnosis of Systemic Sclerosis (VEDOSS) EUSTAR multicentre study: evidence for puffy fingers as a pivotal sign for suspicion of systemic sclerosis. Annals of the rheumatic diseases 2013. 13. Hudson M, Fritzler MJ, Baron M, Canadian Scleroderma Research Group. Systemic sclerosis: establishing diagnostic criteria. Medicine (Baltimore) 2010;89(3):159-65.

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Table 1 Subject characteristics (N=724)

Mean or N

SD or %

Age, years

55.8

12.2

Female

625

86%

White

623

90%

Disease duration, years

10.9

9.0

Limited

424

59%

Diffuse

273

38%

Sine

27

4%

Skin thickening of the fingers of both hands extending proximal to the MCP joints

547

76%

Puffy fingers

453

63%

Sclerodactyly

683

98%

Digital Tip Ulcers

391

54%

Pitting Scars

307

43%

Telangiectasia

524

76%

Abnormal nailfold capillaries

549

76%

Disease subset

PAH*

32

5%

ILD

235

33%

Raynaud’s phenomenon

708

98%

Anti-centromere

262

36%

Anti-topoisomerase

111

15%

Anti-RNA pol III

126

17%

Antibodies

* 8% of subjects were missing adequate data to classify as having or not having PAH.

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Table 2 Comparison between ACR 2013 and 1980 criteria

Subset of patients

New criteria

Old ACR criteria

P -value

N total

N

%

N

%

Entire cohort

724

712

98.3%

639

88.3%

European League against rheumatism classification criteria for systemic sclerosis outperform the 1980 criteria: data from the Canadian Scleroderma Research Group.

The goal of this study was to determine the sensitivity of the new 2013 classification criteria for systemic sclerosis (SSc; scleroderma) in an indepe...
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