Background/Purpose: Little is known about the natural history of primary Sjögren’s syndrome due to the limited number of prospective cohorts followed up in a yearly manner. International validated disease activity scores were recently established and are now used as inclusion criteria and as primary outcome measures in many randomized controlled trials. This is therefore of critical importance to know the evolution of these scores and the proportion of patients experiencing a change in these scores.

Methods: The ASsessment of Systemic complications and Evolution in primary Sjögren’s Syndrome (ASSESS) cohort is a prospective ongoing cohort of 395 patients. All patients have a medical examination every year by a trained physician, who collects the ESSDAI (Eular Sjögren’s Syndrome Disease Activity Index), which scores systemic disease activity, clinical ESSDAI (clin ESSDAI, which scores systemic disease activity without the biological domain of the ESSDAI)and the ESSPRI (Eular Sjögren’s Syndrome Patient Index), a patient-related outcome which scores dryness, pain and fatigue.

Results: Baseline data At enrollment in the 395 patients of the cohort, median ESSDAI, clin ESSDAI and ESSPRI were 3 [2-8], 3 [0-8] and 5.7 [4-7], respectively. 28.2% and 10.6% of patients had moderate systemic disease activity (5≤ ESSDAI < 13) or high systemic disease activity (ESSDAI ≥ 14), respectively. 62.2% of patients had an ESSPRI≥ 5. Follow-up data During the 5 years of annual follow up, 23.3 and 23.4% of patients with a baseline ESSDAI or a clinESSDAI < 5, respectively, had at least at one follow up visit, an ESSDAI or clinESSDAI ≥ 5. 10.5% and 12.9% of patients with a baseline ESSDAI<14 or clinESSDAI<14, respectively, had at least at one follow up visit an ESSDAI or clin ESSDAI≥14. Regarding changes in domains of the ESSDAI over the 5 years of follow up, the most frequent changes were observed in the biological (46.2% of patients), articular (34.9%), hematological (28.9%), pulmonary (25.1%), and glandular domain (20.5%). 25% of patients with a baseline ESSPRI<5 had at least at follow up one visit an ESSPRI ≥5. 39.8% of patients with baseline ESSDAI and ESSPRI<5 had at least at one follow up visit an ESSDAI or an ESSPRI ≥5.

Conclusion: Systemic manifestations and symptoms evolve over time during the 5-year follow-up in patients with primary Sjögren’s Syndrome. Approximately one fourth of the patients with no systemic activity or an acceptable symptoms status at baseline evolved to a status of moderate disease activity or to disabling symptoms, respectively. 40% of patients without any active disease or disabling symptoms at baseline developed systemic complications or disabling symptoms within 5 years.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/evolution-of-disease-activity-over-a-5-year-period-in-the-395-patients-with-primary-sjogrens-syndrome-of-the-assess-prospective-cohort/