2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren's Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts

Caroline H. Shiboski, Stephen C. Shiboski, Raphaèle Seror, Lindsey A. Criswell, Marc Labetoulle, Thomas M. Lietman, Astrid Rasmussen, Hal Scofield, Claudio Vitali, Simon J. Bowman, Xavier Mariette, A. M. Heidenreich, H. Lanfranchi, C. Vollenweider, M. Schiødt, V. Devauchelle, J. E. Gottenberg, A. Saraux, Maggy Pincemin, T. Dörner & 39 others A. Tzoufias, C. Baldini, S. Bombardieri, S. De Vita, K. Kitagawa, T. Sumida, H. Umehara, H. Bootsma, A. A. Kruize, T. R. Radstake, A. Vissink, R. Jonsson, M. Ramos-Casals, E. Theander, S. Challacombe, B. Fisher, B. Kirkham, G. Larkin, F. Ng, S. Rauz, E. Akpek, J. Atkinson, A. N. Baer, S. Carsons, N. Carteron, T. Daniels, B. Fox, J. Greenspan, G. Illei, D. Nelson, A. Parke, S. Pillemer, B. Segal, K. Sivils, E. W. St.Clair, D. Stone, F. Vivino, A. Wu,

Research output: Contribution to journalArticle

Abstract

Objective: To develop and validate an international set of classification criteria for primary Sjögren's syndrome (SS) using guidelines from the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). These criteria were developed for use in individuals with signs and/or symptoms suggestive of SS. Methods: We assigned preliminary importance weights to a consensus list of candidate criteria items, using multi-criteria decision analysis. We tested and adapted the resulting draft criteria using existing cohort data on primary SS cases and non-SS controls, with case/non-case status derived from expert clinical judgment. We then validated the performance of the classification criteria in a separate cohort of patients. Results: The final classification criteria are based on the weighted sum of 5 items: anti-SSA/Ro antibody positivity and focal lymphocytic sialadenitis with a focus score of ≥1 foci/4 mm2, each scoring 3; an abnormal ocular staining score of ≥5 (or van Bijsterveld score of ≥4), a Schirmer's test result of ≤5 mm/5 minutes, and an unstimulated salivary flow rate of ≤0.1 ml/minute, each scoring 1. Individuals with signs and/or symptoms suggestive of SS who have a total score of ≥4 for the above items meet the criteria for primary SS. Sensitivity and specificity against clinician-expert–derived case/non-case status in the final validation cohort were high, i.e., 96% (95% confidence interval [95% CI] 92–98%) and 95% (95% CI 92–97%), respectively. Conclusion: Using methodology consistent with other recent ACR/EULAR-approved classification criteria, we developed a single set of data-driven consensus classification criteria for primary SS, which performed well in validation analyses and are well-suited as criteria for enrollment in clinical trials.

Original languageEnglish (US)
Pages (from-to)35-45
Number of pages11
JournalArthritis and Rheumatology
Volume69
Issue number1
DOIs
StatePublished - Jan 1 2017
Externally publishedYes

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Sjogren's Syndrome
Confidence Intervals
Sialadenitis
Decision Support Techniques
Rheumatology
Rheumatic Diseases
Clinical Trials
Staining and Labeling
Antibodies
Datasets

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology

Cite this

Shiboski, C. H., Shiboski, S. C., Seror, R., Criswell, L. A., Labetoulle, M., Lietman, T. M., ... the International Sjögren's Syndrome Criteria Working Group (2017). 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren's Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Arthritis and Rheumatology, 69(1), 35-45. DOI: 10.1002/art.39859

2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren's Syndrome : A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. / Shiboski, Caroline H.; Shiboski, Stephen C.; Seror, Raphaèle; Criswell, Lindsey A.; Labetoulle, Marc; Lietman, Thomas M.; Rasmussen, Astrid; Scofield, Hal; Vitali, Claudio; Bowman, Simon J.; Mariette, Xavier; Heidenreich, A. M.; Lanfranchi, H.; Vollenweider, C.; Schiødt, M.; Devauchelle, V.; Gottenberg, J. E.; Saraux, A.; Pincemin, Maggy; Dörner, T.; Tzoufias, A.; Baldini, C.; Bombardieri, S.; De Vita, S.; Kitagawa, K.; Sumida, T.; Umehara, H.; Bootsma, H.; Kruize, A. A.; Radstake, T. R.; Vissink, A.; Jonsson, R.; Ramos-Casals, M.; Theander, E.; Challacombe, S.; Fisher, B.; Kirkham, B.; Larkin, G.; Ng, F.; Rauz, S.; Akpek, E.; Atkinson, J.; Baer, A. N.; Carsons, S.; Carteron, N.; Daniels, T.; Fox, B.; Greenspan, J.; Illei, G.; Nelson, D.; Parke, A.; Pillemer, S.; Segal, B.; Sivils, K.; St.Clair, E. W.; Stone, D.; Vivino, F.; Wu, A.; the International Sjögren's Syndrome Criteria Working Group.

In: Arthritis and Rheumatology, Vol. 69, No. 1, 01.01.2017, p. 35-45.

Research output: Contribution to journalArticle

Shiboski, CH, Shiboski, SC, Seror, R, Criswell, LA, Labetoulle, M, Lietman, TM, Rasmussen, A, Scofield, H, Vitali, C, Bowman, SJ, Mariette, X, Heidenreich, AM, Lanfranchi, H, Vollenweider, C, Schiødt, M, Devauchelle, V, Gottenberg, JE, Saraux, A, Pincemin, M, Dörner, T, Tzoufias, A, Baldini, C, Bombardieri, S, De Vita, S, Kitagawa, K, Sumida, T, Umehara, H, Bootsma, H, Kruize, AA, Radstake, TR, Vissink, A, Jonsson, R, Ramos-Casals, M, Theander, E, Challacombe, S, Fisher, B, Kirkham, B, Larkin, G, Ng, F, Rauz, S, Akpek, E, Atkinson, J, Baer, AN, Carsons, S, Carteron, N, Daniels, T, Fox, B, Greenspan, J, Illei, G, Nelson, D, Parke, A, Pillemer, S, Segal, B, Sivils, K, St.Clair, EW, Stone, D, Vivino, F, Wu, A & the International Sjögren's Syndrome Criteria Working Group 2017, '2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren's Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts' Arthritis and Rheumatology, vol 69, no. 1, pp. 35-45. DOI: 10.1002/art.39859

Shiboski, Caroline H.; Shiboski, Stephen C.; Seror, Raphaèle; Criswell, Lindsey A.; Labetoulle, Marc; Lietman, Thomas M.; Rasmussen, Astrid; Scofield, Hal; Vitali, Claudio; Bowman, Simon J.; Mariette, Xavier; Heidenreich, A. M.; Lanfranchi, H.; Vollenweider, C.; Schiødt, M.; Devauchelle, V.; Gottenberg, J. E.; Saraux, A.; Pincemin, Maggy; Dörner, T.; Tzoufias, A.; Baldini, C.; Bombardieri, S.; De Vita, S.; Kitagawa, K.; Sumida, T.; Umehara, H.; Bootsma, H.; Kruize, A. A.; Radstake, T. R.; Vissink, A.; Jonsson, R.; Ramos-Casals, M.; Theander, E.; Challacombe, S.; Fisher, B.; Kirkham, B.; Larkin, G.; Ng, F.; Rauz, S.; Akpek, E.; Atkinson, J.; Baer, A. N.; Carsons, S.; Carteron, N.; Daniels, T.; Fox, B.; Greenspan, J.; Illei, G.; Nelson, D.; Parke, A.; Pillemer, S.; Segal, B.; Sivils, K.; St.Clair, E. W.; Stone, D.; Vivino, F.; Wu, A.; the International Sjögren's Syndrome Criteria Working Group / 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren's Syndrome : A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts.

In: Arthritis and Rheumatology, Vol. 69, No. 1, 01.01.2017, p. 35-45.

Research output: Contribution to journalArticle

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abstract = "Objective: To develop and validate an international set of classification criteria for primary Sjögren's syndrome (SS) using guidelines from the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). These criteria were developed for use in individuals with signs and/or symptoms suggestive of SS. Methods: We assigned preliminary importance weights to a consensus list of candidate criteria items, using multi-criteria decision analysis. We tested and adapted the resulting draft criteria using existing cohort data on primary SS cases and non-SS controls, with case/non-case status derived from expert clinical judgment. We then validated the performance of the classification criteria in a separate cohort of patients. Results: The final classification criteria are based on the weighted sum of 5 items: anti-SSA/Ro antibody positivity and focal lymphocytic sialadenitis with a focus score of ≥1 foci/4 mm2, each scoring 3; an abnormal ocular staining score of ≥5 (or van Bijsterveld score of ≥4), a Schirmer's test result of ≤5 mm/5 minutes, and an unstimulated salivary flow rate of ≤0.1 ml/minute, each scoring 1. Individuals with signs and/or symptoms suggestive of SS who have a total score of ≥4 for the above items meet the criteria for primary SS. Sensitivity and specificity against clinician-expert–derived case/non-case status in the final validation cohort were high, i.e., 96% (95% confidence interval [95% CI] 92–98%) and 95% (95% CI 92–97%), respectively. Conclusion: Using methodology consistent with other recent ACR/EULAR-approved classification criteria, we developed a single set of data-driven consensus classification criteria for primary SS, which performed well in validation analyses and are well-suited as criteria for enrollment in clinical trials.",
author = "Shiboski, {Caroline H.} and Shiboski, {Stephen C.} and Raphaèle Seror and Criswell, {Lindsey A.} and Marc Labetoulle and Lietman, {Thomas M.} and Astrid Rasmussen and Hal Scofield and Claudio Vitali and Bowman, {Simon J.} and Xavier Mariette and Heidenreich, {A. M.} and H. Lanfranchi and C. Vollenweider and M. Schiødt and V. Devauchelle and Gottenberg, {J. E.} and A. Saraux and Maggy Pincemin and T. Dörner and A. Tzoufias and C. Baldini and S. Bombardieri and {De Vita}, S. and K. Kitagawa and T. Sumida and H. Umehara and H. Bootsma and Kruize, {A. A.} and Radstake, {T. R.} and A. Vissink and R. Jonsson and M. Ramos-Casals and E. Theander and S. Challacombe and B. Fisher and B. Kirkham and G. Larkin and F. Ng and S. Rauz and E. Akpek and J. Atkinson and Baer, {A. N.} and S. Carsons and N. Carteron and T. Daniels and B. Fox and J. Greenspan and G. Illei and D. Nelson and A. Parke and S. Pillemer and B. Segal and K. Sivils and St.Clair, {E. W.} and D. Stone and F. Vivino and A. Wu and {the International Sjögren's Syndrome Criteria Working Group}",
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T1 - 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren's Syndrome

T2 - Arthritis and Rheumatology

AU - Shiboski,Caroline H.

AU - Shiboski,Stephen C.

AU - Seror,Raphaèle

AU - Criswell,Lindsey A.

AU - Labetoulle,Marc

AU - Lietman,Thomas M.

AU - Rasmussen,Astrid

AU - Scofield,Hal

AU - Vitali,Claudio

AU - Bowman,Simon J.

AU - Mariette,Xavier

AU - Heidenreich,A. M.

AU - Lanfranchi,H.

AU - Vollenweider,C.

AU - Schiødt,M.

AU - Devauchelle,V.

AU - Gottenberg,J. E.

AU - Saraux,A.

AU - Pincemin,Maggy

AU - Dörner,T.

AU - Tzoufias,A.

AU - Baldini,C.

AU - Bombardieri,S.

AU - De Vita,S.

AU - Kitagawa,K.

AU - Sumida,T.

AU - Umehara,H.

AU - Bootsma,H.

AU - Kruize,A. A.

AU - Radstake,T. R.

AU - Vissink,A.

AU - Jonsson,R.

AU - Ramos-Casals,M.

AU - Theander,E.

AU - Challacombe,S.

AU - Fisher,B.

AU - Kirkham,B.

AU - Larkin,G.

AU - Ng,F.

AU - Rauz,S.

AU - Akpek,E.

AU - Atkinson,J.

AU - Baer,A. N.

AU - Carsons,S.

AU - Carteron,N.

AU - Daniels,T.

AU - Fox,B.

AU - Greenspan,J.

AU - Illei,G.

AU - Nelson,D.

AU - Parke,A.

AU - Pillemer,S.

AU - Segal,B.

AU - Sivils,K.

AU - St.Clair,E. W.

AU - Stone,D.

AU - Vivino,F.

AU - Wu,A.

AU - the International Sjögren's Syndrome Criteria Working Group

PY - 2017/1/1

Y1 - 2017/1/1

N2 - Objective: To develop and validate an international set of classification criteria for primary Sjögren's syndrome (SS) using guidelines from the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). These criteria were developed for use in individuals with signs and/or symptoms suggestive of SS. Methods: We assigned preliminary importance weights to a consensus list of candidate criteria items, using multi-criteria decision analysis. We tested and adapted the resulting draft criteria using existing cohort data on primary SS cases and non-SS controls, with case/non-case status derived from expert clinical judgment. We then validated the performance of the classification criteria in a separate cohort of patients. Results: The final classification criteria are based on the weighted sum of 5 items: anti-SSA/Ro antibody positivity and focal lymphocytic sialadenitis with a focus score of ≥1 foci/4 mm2, each scoring 3; an abnormal ocular staining score of ≥5 (or van Bijsterveld score of ≥4), a Schirmer's test result of ≤5 mm/5 minutes, and an unstimulated salivary flow rate of ≤0.1 ml/minute, each scoring 1. Individuals with signs and/or symptoms suggestive of SS who have a total score of ≥4 for the above items meet the criteria for primary SS. Sensitivity and specificity against clinician-expert–derived case/non-case status in the final validation cohort were high, i.e., 96% (95% confidence interval [95% CI] 92–98%) and 95% (95% CI 92–97%), respectively. Conclusion: Using methodology consistent with other recent ACR/EULAR-approved classification criteria, we developed a single set of data-driven consensus classification criteria for primary SS, which performed well in validation analyses and are well-suited as criteria for enrollment in clinical trials.

AB - Objective: To develop and validate an international set of classification criteria for primary Sjögren's syndrome (SS) using guidelines from the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). These criteria were developed for use in individuals with signs and/or symptoms suggestive of SS. Methods: We assigned preliminary importance weights to a consensus list of candidate criteria items, using multi-criteria decision analysis. We tested and adapted the resulting draft criteria using existing cohort data on primary SS cases and non-SS controls, with case/non-case status derived from expert clinical judgment. We then validated the performance of the classification criteria in a separate cohort of patients. Results: The final classification criteria are based on the weighted sum of 5 items: anti-SSA/Ro antibody positivity and focal lymphocytic sialadenitis with a focus score of ≥1 foci/4 mm2, each scoring 3; an abnormal ocular staining score of ≥5 (or van Bijsterveld score of ≥4), a Schirmer's test result of ≤5 mm/5 minutes, and an unstimulated salivary flow rate of ≤0.1 ml/minute, each scoring 1. Individuals with signs and/or symptoms suggestive of SS who have a total score of ≥4 for the above items meet the criteria for primary SS. Sensitivity and specificity against clinician-expert–derived case/non-case status in the final validation cohort were high, i.e., 96% (95% confidence interval [95% CI] 92–98%) and 95% (95% CI 92–97%), respectively. Conclusion: Using methodology consistent with other recent ACR/EULAR-approved classification criteria, we developed a single set of data-driven consensus classification criteria for primary SS, which performed well in validation analyses and are well-suited as criteria for enrollment in clinical trials.

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