Objectives To characterize and quantify systemic involvement at diagnosisinalarge international cohort of patients diagnosed with primary Sjogren syndrome (SS).
Methods The Big Data Sjögren Project is an international, multicenter registry formed in 2014 with the aim of taking a “high-definition” picture of the main features of primary SS at diagnosis by merging international SS databases. By January 2015, the database included 5027 consecutive patients fulfilling the 2002 classification criteria for primary SS from 13 countries (9 European, 4 American). Systemic involvement was defined according to the ESSDAI and retrospectively calculated.
Results Baseline ESSDAI data was collected in3314 patients (94% female, mean age at diagnosis 54.25 years). The main features of systemic involvement at diagnosis included biological activity in 43%of patients, articular involvement in 35%, hematological activity in 25%and glandular involvementin 19%. The mean ESSDAI score at diagnosis of the entire cohort was 5.63 (range, 0-62). Low DAS was reported in 1267 (38%) patients, moderate DASin 943 (28%) and high DAS in 378 (11%) patients; in the remaining 726 patients (22%), the ESSDAI score at diagnosis was 0. The mean baseline ESSDAI was higher in males (7.33 vs 5.52, p<0.001), patients diagnosed ≤35 years (6.71 vs 5.63, p=0.001), those with positive ocular tests (5.75 vs 4.98, p=0.023) and those with positive immunological markers including ANA (6.71 vs 4.63, p<0.001), RF (7.46 vs 5.49, p<0.001), anti-Ro/SSA (6.77 vs 5.48, p<0.001) and anti-La/SSB (6.91 vs 6.0, p<0.001). According to the number of criteria fulfilled at diagnosis, a higher mean baseline ESSDAI was found in patients fulfilling the six criteria (7.67 vs 5.65 in those fulfilling 5 criteria and 5.23 in those fulfilling 3-4 criteria, p<0.001).
Conclusions Epidemiological features (male sex, younger age at diagnosis) and positive autoantibodies at diagnosis were closely-related to greater systemic activity. Using the ESSDAI in real-life situations may help identify epidemiological and immunological subsets with high systemic activity at diagnosis and, therefore, at high risk of suffering a complicated clinical course.
Disclosure of Interest None declared