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.
(2017) Arthritis & Rheumatology, volume 69, issue 1, pp. 35 - 45
(Article)
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
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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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Keywords: Immunology and Allergy, Rheumatology, Immunology, Journal Article, Consensus Development Conference, Validation Studies
ISSN: 2326-5191
Publisher: John Wiley & Sons Inc.
Note: Funding Information: We would like to express our appreciation to Steve Taylor and Kathy Hammitt (Sjögren's Syndrome Foundation) for hosting 3 of the meetings of the International SS Criteria Working Group, Dr. Frédéric Desmoulins for his important work in preparation of the Paris-Sud cohort data set, and Mi Lam for her contribution in preparation of the SICCA data set. We are very grateful to Paul Hansen and Franz Ombler, the developers and owners of the 1000Minds software (https://www.1000minds.com), who granted us an Academic Award, providing both access to and technical support for their software. We also express our greatest appreciation to all participants who enrolled in the 3 patient cohorts used for development and validation of the criteria, and to the clinician-expert members of the international working group for attending meetings, providing valuable input as part of these meetings, and responding to several rounds of surveys, including grading multiple vignettes. Publisher Copyright: © 2016 The Authors. Arthritis & Rheumatology published by Wiley Periodicals, Inc. on behalf of the American College of Rheumatology.
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