2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren's syndrome

TLDR

The criteria were intended for individuals exhibiting signs or symptoms suggestive of primary Sjögren's syndrome. The study aimed to develop and validate an international set of classification criteria for primary Sjögren's syndrome using ACR and EULAR guidelines. The authors weighted candidate criteria items via multi‑criteria decision analysis, then tested and refined the draft criteria on existing cohort data and validated the final set in an independent cohort. The final criteria comprise a weighted sum of five items—anti‑SSA/Ro positivity, focal lymphocytic sialadenitis (focus score ≥ 1), abnormal ocular staining (≥ 5 or van Bijsterveld ≥ 4), Schirmer test ≤ 5 mm/5 min, and unstimulated salivary flow ≤ 0.1 mL/min—requiring a total score ≥ 4, yielding 96 % sensitivity and 95 % specificity, and are suitable for clinical trial enrolment.

Abstract

<h3>Objectives</h3> To develop and validate an international set of classification criteria for primary Sjögren9s 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. <h3>Methods</h3> 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 judgement. We then validated the performance of the classification criteria in a separate cohort of patients. <h3>Results</h3> The final classification criteria are based on the weighted sum of five 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 Schirmer9s test result of ≤5 mm/5 min and an unstimulated salivary flow rate of ≤0.1 mL/min, 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, that is, 96% (95% CI92% to 98%) and 95% (95% CI 92% to 97%), respectively. <h3>Conclusion</h3> 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 enrolment in clinical trials.