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FRI0419 Big Data Sjogren Project (Eular-SS Task Force International Network): Systemic Involvement at Diagnosis Evaluated by the Essdai in 3314 Patients with Primary Sjögren Syndrome
  1. P. Brito Zeron1,
  2. B.A. Kostov2,
  3. R. Seror3,
  4. C. Baldini4,
  5. L. Quartuccio5,
  6. M. Kvarnstrom6,
  7. A. Kruize7,
  8. G. Hernández Molina8,
  9. S. Praprotnik9,
  10. E. Bartoloni10,
  11. R. Solans11,
  12. E. Theander12,
  13. V. Valim13,
  14. R. Priori14,
  15. M. Zeher15,
  16. D. Isenberg16,
  17. A. Rasmussen17,
  18. R. Giacomelli18,
  19. S. Carsons19,
  20. D. Hammenfors20,
  21. C. Vollenweider21,
  22. F. Atzeni22,
  23. T. Mandl12,
  24. S. De Vita5,
  25. M. Wahren-Herlenius6,
  26. J. Sanchez-Guerrero23,
  27. R. Gerli10,
  28. K. Sivils17,
  29. S. Mowa19,
  30. J.G. Brun20,
  31. X. Mariette24,
  32. M. Ramos-Casals1
  33. on behalf of EULAR-SS Task Force International Network
  1. 1Hosp. Clinic, IDIBAPS
  2. 2CAPSBE, IDIBAPS, Barcelona, Spain
  3. 3Hosp. Hotel Dieu, Paris, France
  4. 4Rheumatol Clinic, Pisa
  5. 5Univ. Hosp. Santa Maria Misericordia, Udine, Italy
  6. 6Karolinska Inst, Stockholm, Sweden
  7. 7UMCU, Utrecht, Netherlands
  8. 8INCMyN Salvador Zubirán, Mexico, Mexico
  9. 9Univ. Clin Center, Ljubljana, Slovenia
  10. 10Univ. Perugia, Perugia, Italy
  11. 11Hosp Vall Hebron, Barcelona, Spain
  12. 12Lund Univ., Malmo, Sweden
  13. 13Univ Fed Espírito Santo, Vitoria, Brazil
  14. 14Sapienza Universita, Roma, Italy
  15. 15Univ. Debrecen, Debrecen, Hungary
  16. 16UCL, London, United Kingdom
  17. 17OMRF, Oklahoma, United States
  18. 18Univ of, L'Aquila, Italy
  19. 19Stony Brook Univ. School, Mineola N.Y., United States
  20. 20Haukeland Univ Hosp, Bergen, Norway
  21. 21German Hosp, Buenos Aires, Argentina
  22. 22Sacco Univ Hosp, Milan, Italy
  23. 23Mount Sinai Hosp, Toronto, Canada
  24. 24Université Paris-Sud, Paris, France

Abstract

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

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