ReviewOutcome measures for primary Sjögren's syndrome: A comprehensive review
Section snippets
Assessment of systemic disease activity: the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI)
For evaluation of systemic activity, the SSDAI was the first activity index proposed for SS. It offered the advantage of great simplicity, but the major disadvantage is of a lack of exhaustiveness, which results in an important floor effect with more than half of the patients having a score of 0 or 1 in a real life cohort of primary SS patients [26]. By contrast, the SCAI is much more exhaustive, but is so complex to rate that it is very difficult to use in clinical practice. Compared to
EULAR validation study
Both ESSDAI and ESSPRI have been recently validated in a prospective international cohort that included 395 patients in 15 countries [29]. The aim of this study was to evaluate psychometric properties (construct validity, responsiveness and reliability) and compare them to that of previous scores: SSDAI and SCAI for ESSDAI and SSI and PROFAD for ESSPRI. Like all scores, EULAR scores had excellent reliability. However, they have been found to have better construct validity (i.e. higher
Future steps: conducting clinical trials
The objective is now for these indexes to be used as outcome criteria in randomized controlled trial, in order to be able to demonstrate, if any, efficacy of treatments. Effectively, the treatment of pSS remains a challenge, since most of randomized controlled trials, even the most recent ones, failed to demonstrate efficacy of the evaluated treatment. Hydroxychloroquine which is the most frequently proposed treatment, is used based on observational studies [49] and one cross-over study that
Conclusion
We have now two validated indexes to completely assess disease features of primary SS patients. These tools are complementary and should be used together in addition to objectives measures of dryness and biological markers of activity. Depending on the component of disease that is the target of the treatment, ESSDAI or ESSPRI should be outcome measure of clinical trials. Work is currently in progress to better define disease activity levels and response criteria using these indexes. This
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On behalf of the EULAR Sjögren's Task Force.