Skip to main content

Advertisement

Log in

ESSDAI activity index of the SJÖGRENSER cohort: analysis and comparison with other European cohorts

  • Cohort Studies
  • Published:
Rheumatology International Aims and scope Submit manuscript

Abstract

The objective of the study was to assess the ESSDAI index characteristics in the SJÖGRENSER cohort (Spanish Rheumatology Association’s registry of patients with Primary Sjögren Syndrome [PSS]). SJÖGRENSER is a prospective multicentric study on a cohort of Spanish patients with PSS who meet the 2002 American–European consensus from rheumatology units. 298 variables were studied in patients for the inclusion of the study from an anonymous list from each department. The ESSDAI (EULAR Sjögren’s syndrome disease activity index) includes 12 domains and measures systematic activity in PSS patients. Each domain is divided into 3–4 levels, (0: no activity; 1: low activity; 2: moderate activity; 3: high activity) and is attributed a weight. Each domain score is obtained by multiplying the activity level by the weight assigned. According to ESSDAI: low activity < 5; moderate activity 5–13, and high activity ≥ 14. ESSDAI was compared between several European PSS cohorts (EULAR, ASSES, GEAS, GRISS, Ducth). 437 patients were included from 33 Spanish rheumatology units. 95.2% were women with a median age of 58.63 years [p25–p75: 50.02–67.98 years] and average PSS evolution of 10.4 years (6–16 years). ESSDAI median on entering the study was 2 (0–4). 31% of patients had ESSDAI 0; low activity 49%, moderate activity 15%, and high activity 5%. Those with greater activity were the joint, haematological and biological domains, whereas the lung was the most affected organ with pleural and parenchymatous involvement. Unlike other European cohorts, the initial SJÖGRENSER cohort was characterised by low-zero systemic activity in 80% of patients, which differentiates it from other cohorts and provides a prospective study opportunity.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. FoxX RI (2005) Sjögren’s syndrome. Lancet 366:321–331

    Article  CAS  Google Scholar 

  2. Moutsopoulos (1994) Sjögren’s syndrome: autoimmune epithelitis. Clin Immunol Immunopathol 72:162–165

    Article  CAS  PubMed  Google Scholar 

  3. Aagyropoulou O, Valentini E, Ferro F et al (2018) One year in review 2018: Sjögren's syndrome. Clin Exp Rheumatol 36(Suppl 112):27–28

    Google Scholar 

  4. Qin B, Wang J, Yang Z et al (2015) Epidemiology of primary Sjögren’s syndrome: A systematic review and meta-analysis. Ann Rheum Dis 74:1983–1989

    Article  CAS  PubMed  Google Scholar 

  5. Seror R, Ravaud P, Bowman SJ et al (2010) EULAR Sjögren’s syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjögren’s syndrome. Ann Rheum Dis 69:1103–1109

    Article  PubMed  Google Scholar 

  6. Seror R, Bootsma H, Saraux A et al (2016) Defining disease activity states and clinically meaningful improvement in primary Sjögren’s syndrome with EULAR primary Sjögren’s syndrome disease activity (ESSDAI) and patient-reported indexes (ESSPRI). Ann Rheum Dis 75:382–389

    Article  CAS  PubMed  Google Scholar 

  7. Souza FB, Porfirio GJ, Andriolo BN et al (2016) Rituximab effectiveness and safety for treating primary Sjögren’s Syndrome (pSS): systematic review and meta-analysis. PLoS One 11:e0150749

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Carubbi F, Cipriani P, Marelli A et al (2013) Efficacy and safety of rituximab treatment in early primary Sjögren’s syndrome: a prospective, multi-center, follow-up study. Arthritis Res Ther 15:R172

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Mekinian A, Ravaud P, Hatron PY et al (2012) Efficacy of rituximab in primary Sjögren’s syndrome with peripheral nervous system involvement: results from the AIR registry. Ann Rheum Dis 71:84–87

    Article  CAS  PubMed  Google Scholar 

  10. Gottenberg JE, Cinquetti G, Larroche C et al (2013) Efficacy of rituximab in systemic manifestations of primary Sjögren’s syndrome: results in 78 patients of the autoimmune and Rituximab registry. Ann Rheum Dis 72:1026–1031

    Article  CAS  PubMed  Google Scholar 

  11. Meiners PM, Arends S, Brouwer E et al (2012) Responsiveness of disease activity indices ESSPRI and ESSDAI in patients with primary Sjögren’s syndrome treated with rituximab. Ann Rheum Dis 71:1297–1302

    Article  CAS  PubMed  Google Scholar 

  12. Seror R, Mariette X, Bowman S et al (2010) Accurate detection of changes in disease activity in primary Sjögren’s syndrome by the European League Against Rheumatism Sjögren’s Syndrome Disease Activity Index. Arthritis Care Res (Hoboken) 62:551–558

    Article  Google Scholar 

  13. Ramos-Casals M, Solans R, Rosas J et al (2008) Primary Sjögren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine 87:210–219

    Article  CAS  PubMed  Google Scholar 

  14. Vitali C, Bombardieri S, Jonsson R et al (2002) Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 61:554–558

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Fernández Castro M, Andreu JL, Sánchez-Piedra C et al (2016) Sjögren-SER: National registry of the Spanish Society of Rheumatology of patients with primary Sjögren syndrome: objectives and methodology. Reumatol Clin 12:184–189

    Article  PubMed  Google Scholar 

  16. Seror R, Bowman SJ, Brito-Zerón P et al (2015) EULAR Sjögren’s syndrome disease activity index (ESSDAI): a user guide. RMD Open 1:e000022

    Article  PubMed  PubMed Central  Google Scholar 

  17. Gottenberg JE, Seror R, Micelli-Richard C et al (2013) Serum levels of Beta2-microglobulin and free light chains of immunoglobulins are associated with systemic disease activity in primary Sjögren’s Syndrome. Data at enrollment in the prospective ASSESS Cohort. PLoS One 8:e59868. https://doi.org/10.1371/journal.pone.0059868

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Ramos-Casals M, Brito-Zerón P, Solans R et al (2014) Systemic involvement in primary Sjögren’s syndrome evaluated by the EULAR-SS disease activity index: analysis of 921 Spanish patients (GEAS-SS Registry). Rheumatology 53:321–331

    Article  PubMed  Google Scholar 

  19. Quartuccio L, Baldini C, Priori R et al (2017) Cryoglobulinemia in Sjögren's Syndrome: a disease subset that links higher systemic disease activity, autoimmunity, and local B cell proliferation in mucosa-associated lymphoid tissue. J Rheumatol 44:1179–1183

    Article  PubMed  Google Scholar 

  20. Risselada AO, Kruize AA, Bijlsma JWJ (2012) Clinical applicability of the EULAR Sjögren’s syndrome disease activity index: a cumulative ESSDAI score adds in describing disease severity. Ann Rheum Dis 71:631–632

    Article  PubMed  Google Scholar 

  21. Moerman RV, Arends S, Meiners PM et al (2014) EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI) is sensitive to show efficacy of rituximab treatment in a randomized controlled trial. Ann Rheum Dis 73:472–474

    Article  CAS  PubMed  Google Scholar 

  22. Mariette X, Roux S, Zhang J et al (2003) The level of BLyS (BAFF) correlates with the titer of autoantibodies in human Sjögren’s syndrome. Ann Rheum Dis 62:168–171

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. De Vita S, Quartuccio L, Seror R et al (2015) Efficacy and safety of belimumab given for 12 months in primary Sjögren’s syndrome: the BELISS open-label phase II study. Rheumatology. (Oxford) 54:2249–2256

    Google Scholar 

  24. Quartuccio L, Salvin S, Corazza L et al (2016) Efficacy of belimumab and targeting of rheumatoid factor-positive B-cell expansion in Sjögren’s syndrome: follow-up after the end of the phase II open-label BELISS study. Clin Exp Rheumatol 34:311–314

    PubMed  Google Scholar 

  25. Meiners PM, Vissink A, Kroese FG et al (2014) Abatacept treatment reduces disease activity in early primary Sjögren’s syndrome (open-label proof of concept ASAP study). Ann Rheum Dis 73:1393–1396

    Article  CAS  PubMed  Google Scholar 

  26. Dumusc A, Wan-Fai NG, James K et al (2018) Comparison of ESSDAI and ClinESSDAI in potential optimization of trial outcomes in primary Sjögren’s syndrome: examination of data from the UK Primary Sjögren’s Syndrome Registry. Swiss Med Wkly 148:w14588

    PubMed  Google Scholar 

Download references

Acknowledgements

Collaborating researchers SJÖGRENSER group: Raúl Menor (Hospital General Jerez de la Frontera, Jerez de la Frontera). Beatriz Rodríguez (Hospital Universitario de Canarias, Santa Cruz de tenerife). Ángel García Aparicio (Hospital Virgen de la Salud, Toledo). Francisco Javier López Longo (Hospital Gregorio Marañón, Madrid). Sara Manrique-Arija (Hospital Carlos Haya, Málaga). Jesús Alberto García Vadillo (Hospital de la Princesa, Madrid). Susana Gil Barato (Hospital General de Alicante, Alicante). Ruth López-González (Hospital Virgen de la Concha, Zamora). Francisco Javier Narváez (Hospital de Bellvitge, Barcelona). Carlos Galisteo (Hospital Parc-Taulí, Sabadell). Jorge González Martín (Hospital Madrid Norte Sanchinarro, Madrid). Esther Ruiz Lucea (Hospital de Basurto, Basurto). Antonio Naranjo, Celia Erausquin, Iñigo Rúa-Figueroa (Hospital Doctor Negrín, Las Palmas de Gran Canaria). Óscar Illera (Hospital Infanta Sofía, Madrid). Lurdes Romani (Hospital Virgen de las Nieves, Granada). Sheila Melchor (Hospital Doce de Octubre, Madrid). Begoña Moreira (Hospital de Sierrallana, Torrelavega). Enrique Raya (Hospital Clínico San Cecilio, Granada). Marina Rodríguez López, Coral Mouriño, Jose María Pego (Hospital de Meixoeiro, Vigo). Natalia Cid (Hospital de Valme, Sevilla). Enrique Júdez (Hospital de Albacete, Albacete). Clara Moriano (Hospital de León, León). Vicente Torrente (Hospital de L’Hospitalet, Hospitalet de Llobregat). Hèctor Corominas (Hospital Sant Joan Despí Moisès Broggi, Barcelona). Blanca García Magallón (Hospital Miguel Servet, Zaragoza). Carlos Guillén Astete (Hospital Ramón y Cajal, Madrid). Ivan Castellvi (Hospital San Pau y Santa Creu, Barcelona). Cristina Bohórquez (Hospital Príncipe de Asturias, Alcalá de Henares), Javier Loricera (Hospital Marqués de Valdecilla, Santander), Joaquín Belzunegui (Hospital de Donostia, Gipuzkoa), Tatiana Cobo (Hospital Infanta Sofia, Madrid). Spain.

Author information

Authors and Affiliations

Authors

Consortia

Corresponding author

Correspondence to José Rosas.

Ethics declarations

Conflict of interest

None of the authors declare that they have conflict of interest in relation to this manuscript.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

The members of the institutional author SJÖGRENSER Group, part of the Spanish Society of Rheumatology Systemic Autoimmune Diseases Study Group (EASSER), are mentioned in Acknowledgements section.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Rosas, J., Sánchez-Piedra, C., Fernández-Castro, M. et al. ESSDAI activity index of the SJÖGRENSER cohort: analysis and comparison with other European cohorts. Rheumatol Int 39, 991–999 (2019). https://doi.org/10.1007/s00296-019-04285-w

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00296-019-04285-w

Keywords

Navigation