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OP0089 Big Data Sjögren Project (Eular-SS Task Force International Network): Characterization at Diagnosis of 5027 Patients with Primary Sjögren Syndrome
  1. P. Brito Zeron1,
  2. B.A. Kostov2,
  3. M. Zeher3,
  4. E. Theander4,
  5. J.-E. Gottenberg5,
  6. C. Baldini6,
  7. L. Quartuccio7,
  8. R. Priori8,
  9. M. Kvarnstrom9,
  10. A. Kruize10,
  11. G. Hernández Molina11,
  12. S. Praprotnik12,
  13. D. Isenberg13,
  14. E. Bartoloni14,
  15. A. Rasmussen15,
  16. R. Solans16,
  17. V. Valim17,
  18. R. Giacomelli18,
  19. S. Carsons19,
  20. D. Hammenfors20,
  21. C. Vollenweider21,
  22. F. Atzeni22,
  23. T. Mandl4,
  24. S. De Vita7,
  25. M. Wahren-Herlenius9,
  26. J. Sanchez-Guerrero23,
  27. R. Gerli14,
  28. K. Sivils15,
  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. 3Univ. Debrecen, Debrecen, Hungary
  4. 4Lund Univ., Malmo, Sweden
  5. 5Hospitaux Univ., Strasbourg, France
  6. 6Rheumatol Clinic, Pisa
  7. 7Univ. Hosp. Santa Maria Misericordia, Udine
  8. 8Sapienza Univ. di Roma, Roma, Italy
  9. 9Karolinska Inst, Stockholm, Sweden
  10. 10UMCU, Utrecht, Netherlands
  11. 11INCMyN Salvador Zubirán, Mexico, Mexico
  12. 12Univ. Clin Center, Ljubljana, Slovenia
  13. 13UCL, London, United Kingdom
  14. 14Univ. Perugia, Perugia, Italy
  15. 15OMRF, Oklahoma, United States
  16. 16Hosp Vall Hebron, Barcelona, Spain
  17. 17Univ Fed Espírito Santo, Vitoria, Brazil
  18. 18University 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. 24Univ. Paris-Sud, Paris, France

Abstract

Objectives To analyse the epidemiological, clinical and immunological characteristics of an international cohort of patients diagnosed with primary Sjögren syndrome (SS) according to the 2002 AE criteria.

Methods The Big Data Sjögren Project is an international, multicentre registry formed in 2014 to take a “high-definition” picture of the main features of primary SS at diagnosis by merging international SS databases. By January 2015, 5027 consecutive patients fulfilling the 2002 classification criteria for primary SS were included from 9 European and 4 American countries.

Results The cohort included 4714 (94%) women (female:male ratio, 15:1), with a mean age at diagnosis of primary SS of 54 years (range, 10-97), of which 94% were Caucasian and 88% lived in European countries. The frequency of fulfilment of the 2002 criteria was: 94.4% for dry eye, 92.9% for dry mouth, 88.5% for positive salivary gland biopsy, 85.9% for positive ocular tests, 74.8% for positive oral tests and 70.9% for positive Ro/La autoantibodies. As a minimum of 4 of the 6 criteria are required for fulfilment, the percentage of diagnostic tests performed varied: Ro/La autoantibodies were tested in 99.5% of patients, ocular diagnostic tests (Schirmer's test and/or corneal stainings) were made in 90.2%, oral tests in 76.7% and salivary gland biopsy in 72% of patients. Systemic involvement at diagnosis was retrospectively measured using ESSDAI definitions in 3314 patients and included articular involvement (35.2%), glandular involvement (19.3%), lymphadenopathy (10.7%), cutaneous involvement (9%), constitutional involvement (8.4%), respiratory involvement (7.3%), peripheral nervous system involvement (5.2%), renal involvement (2.2%), central nervous system involvement (1.7%) and muscular involvement (1.2%). With respect to laboratory abnormalities, 43.3% of patients showed biological abnormalities and 24.7% haematological abnormalities according to ESSDAI definitions.

Conclusions In this international cohort of 5027 patients with primary SS, most non-sicca clinical features and laboratory abnormalities present at diagnosis are not included in the current criteria; their inclusion in future proposed classification criteria should be evaluated.

Disclosure of Interest None declared

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