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THU0284 Predicting survival in 6240 patients with primary sjÖgren' syndrome (big data sjÖgren project)
  1. P Brito-Zerόn1,2,
  2. N Acar-Denizli3,
  3. M Zeher4,
  4. A Rasmussen5,
  5. X Li6,
  6. C Baldini7,
  7. J-E Gottenberg8,
  8. D Danda9,
  9. L Quartuccio10,
  10. G Hernandez-Molina11,
  11. AA Kruize12,
  12. S-H Park13,
  13. M Kvarnström14,
  14. S Praprotnik15,
  15. D Sene16,
  16. E Bartoloni17,
  17. R Solans18,
  18. T Mandl19,
  19. Y Suzuki20,
  20. M Rischmueller21,
  21. G Nordmark22,
  22. G Fraile23,
  23. A Sebastian24,
  24. H Bootsma25,
  25. T Nakamura26,
  26. V Valim27,
  27. R Giacomelli28,
  28. R Seror29,
  29. V Devauchelle-Pensec30,
  30. B Hofauer31,
  31. M Bombardieri32,
  32. V Trevisani33,
  33. D Hammenfors34,
  34. R Priori35,
  35. SG Pasoto36,
  36. J Morel37,
  37. S Retamozo38,
  38. TA Gheita39,
  39. F Atzeni40,
  40. C Vollenveider41,
  41. X Mariette29,
  42. M Ramos-Casals2,
  43. on behalf of the EULAR-SS Task Force Big Data Consortium
  1. 1Hosp CIMA-Sanitas
  2. 2Hosp Clínic, Barcelona, Spain
  3. 3Msgsü, Istanbul, Turkey
  4. 4Univ, Debrecen, Hungary
  5. 5OMRF, Oklahoma, United States
  6. 6Anhui Hosp, Hefei, China
  7. 7Univ, Pisa, Italy
  8. 8Univ, Strasbourg, France
  9. 9CMC, Vellore, India
  10. 10Santa Maria, Udine, Italy
  11. 11INCMNSZ, México, Mexico
  12. 12UMC, Utrecht, Netherlands
  13. 13Catholic Univ, Seoul, Korea, Republic Of
  14. 14Karolinska Instit, Stockholm, Sweden
  15. 15UMCL, Ljubljana, Slovenia
  16. 16Lariboisière Hosp, Paris, France
  17. 17Univ, Perugia, Italy
  18. 18Hosp Vall Hebron, Barcelona, Spain
  19. 19Lund Univ, Malmö, Sweden
  20. 20Univ, Kanazawa, Japan
  21. 21TQEH, Adelaide, Australia
  22. 22Univ, Uppsala, Sweden
  23. 23Hosp Ramόn Cajal, Madrid, Spain
  24. 24Med Hosp, Wroclaw, Poland
  25. 25Univ, Groningen, Netherlands
  26. 26Univ, Nagasaki, Japan
  27. 27UFES, Vitόria, Brazil
  28. 28Univ, L'Aquila, Italy
  29. 29Univ Paris Sud, Paris
  30. 30Univ, Brest, France
  31. 31TUM, München, Germany
  32. 32QMUL, London, United Kingdom
  33. 33UNIFESP, Sao Paulo, Brazil
  34. 34Haukeland Hosp, Bergen, Norway
  35. 35Sapienza Univ, Rome, Italy
  36. 36USP, Sao Paulo, Brazil
  37. 37Univ, Montpellier, France
  38. 38INICSA, Cordoba, Argentina
  39. 39Univ, Cairo, Egypt
  40. 40L.Sacco Univ Hosp, Milan, Italy
  41. 41German Hosp, Buenos Aires, Argentina


Objectives To correlate the main features at diagnosis with survival in a multicenter international cohort of patients with primary Sjögren syndrome (SS).

Methods The Big Data Sjögren Project was formed in 2014 to take a “high-definition” picture of the main features of primary SS by merging international SS databases (9302 consecutive patients recruited from 21 countries of the 5 continents)

Results Data about survival at the last visit was available in 6240 patients (5831 women, mean age at diagnosis of 53yrs, 82% White). After a mean follow-up of 7.1yrs, 407 (6%) patients died. Patients who died were more frequently men (10% vs 6%, p=0.004) and White (96% vs 81%, p<0.001), had a higher mean age at diagnosis (64 vs 52yrs. p<0.001) and a higher frequency of abnormal ocular (95% vs 87%, p<0.001) and oral (90% vs 79%, p<0.001) tests in comparison with survivors; sicca symptoms and salivary gland biopsy were unrelated to survival. Immunologically, patients who died showed a higher frequency of RF (52% vs 46%, p=0.039) and cryoglobulins (18% vs 7%, p<0.001). Logistic regression identified as independent variables the age at diagnosis (OR 1.07), male gender (OR 1.77), abnormal ocular (OR 5.79) and oral (OR 2.10) tests, and cryoglobulins (OR 3.10). Time-adjusted multivariate Cox proportional-hazards regression analysis confirmed age at diagnosis (OR 1.12, CI95% 1.10–1.14), male gender (OR 2.12, CI95% 1.36–3.29) and cryoglobulins (OR 2.43, CI95% 1.72–3.42) as independent variables related to death.

Conclusions An older age at diagnosis, male gender and serum cryoglobulins were the principal risk factors associated with death in this large multiethnic cohort of patients with primary SS.

Disclosure of Interest None declared

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