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SAT0417 Cancer and Primary SjÖgren Syndrome in 1216 Patients (GEAS-SS Registry): Systemic Activity Measured by the Essdai is Related to Hematological, but not Solid Neoplasia
  1. P. Brito Zeron1,
  2. B. Kostov2,
  3. R. Solans3,
  4. G. Fraile4,
  5. A. García-Pérez5,
  6. B. Maure6,
  7. F.-J. Rascόn7,
  8. M. Lopez-Dupla8,
  9. M. Ripoll9,
  10. C. Lόpez González-Cobos10,
  11. E. Fonseca11,
  12. M. Akasbi12,
  13. M. Pérez-de-Lis13,
  14. I. Jiménez-Heredia14,
  15. G. de la Red15,
  16. A. Gato16,
  17. M. Ramentol3,
  18. A. Ruedas4,
  19. B. Díaz-Lόpez5,
  20. L. Pallarés7,
  21. H. Gheitasi1,
  22. M. Ramos-Casals1
  1. 1Laboratory of Autoimmune Diseases Josep Font, IDIBAPS, Department of Autoimmune Diseases, ICMiD, Hospital Clínic
  2. 2Primary Care Research Group, IDIBAPS, Centre d'Assistència Primària ABS Les Corts, Gesclinic
  3. 3Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona
  4. 4Department of Internal Medicine, Hospital Ramόn y Cajal, Madrid
  5. 5Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo
  6. 6Department of Internal Medicine, Complejo Hospitalario Universitario, Vigo
  7. 7Department of Internal Medicine, Hospital Son Espases, Palma de Mallorca
  8. 8Department of Internal Medicine, Hospital Joan XXIII, Tarragona
  9. 9Department of Internal Medicine, Hospital Infanta Sofía
  10. 10Department of Internal Medicine, Hospital Gregorio Marañόn, Madrid
  11. 11Department of Internal Medicine, Hospital de Cabueñes, Gijόn
  12. 12Department of Internal Medicine, Hospital Infanta Leonor, Madrid
  13. 13Department of Internal Medicine, Hospital do Meixoeiro, vigo
  14. 14Department of Internal Medicine, Hospital de Sagunto, Valencia
  15. 15Department of Internal Medicine, Hospital Esperit Sant, Badalona
  16. 16Department of Internal Medicine, Complejo Hospitalario Albacete, Albacete, Spain


Objectives To analyse the epidemiological, clinical and analytical features at diagnosis and the use of the European systemic activity index (ESSDAI) to predict the development of cancer in a large cohort of Spanish patients with primary Sjogren syndrome (SS).

Methods The GEAS-SS multicenter registry was formed in 2005 with the aim of collecting a large series of Spanish patients with primary SS, and included thirteen Spanish reference centers with substantial experience in the management of SS patients. By January 2015, the database included 1216 consecutive patients fulfilling the 2002 classification criteria for primary SS. ESSDAI scores were retrospectively calculated at diagnosis. Disease activity states (DAS) were defined according to the baseline ESSDAI score (low DAS for an ESSDAI <4, moderate DAS for an ESSDAI between 5 and 13, and high DAS for an ESSDAI higher than 13). Cancer was categorized as hematological or solid neoplasia.

Results After a mean follow-up of 114 months, 84 (7%) patients developed solid cancer and 52 (4%) hematological neoplasia. The following baseline features at diagnosis were associated with the development of hematological neoplasia: male gender (19% vs 6%, p<0.001), age at diagnosis (59 vs 54 yrs, p<0.001), baseline systemic activity (75% vs 51%, p=0.001), neutropenia (19% vs 9%, p=0.028), C3 values <0.82 g/L (19% vs 10%, p=0.048), C4 values <0.07 g/L (24% vs 12%, p=0.02), monoclonal serum band (27% vs 8%, p<0.001) and cryoglobulins (30% vs 9%, p<0.001). In contrast, only age (59 vs 54 yrs, p<0.001) and neutropenia (21% vs 9%, p=0.001) were related to the development of solid neoplasia. High systemic activity (high-DAS) was found in a higher frequency in patients who developed hematological in comparison with those who developed solid neoplasia or those without neoplasia (79% vs 24% vs 21%, p<0.001).

Conclusions Systemic and biological activity is closely related to the development of lymphoma in primary SS. Etiopathogenic factors such as B-cell hyperactivity and monoclonal, cryoglobulinemic-driven immunological responses have a dual effect, enhancing the risk of development of both systemic involvement and hematological neoplasia, but not the risk of solid neoplasia, whose development is independent of systemic Sjögren.

Disclosure of Interest None declared

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