Skip to main content
SpringerLink
Log in

Advances in the diagnosis and treatment of Sjogren’s syndrome

  • Review Article
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Sjogren’s syndrome (SS) is a systemic autoimmune disease that primarily affects the exocrine glands, resulting in dryness of the eyes and mouth due to lymphocytic infiltration of the salivary and lacrimal glands along with arthritis, kidney, liver, and lung involvement, chronic fatigue, musculoskeletal pain, vasculitis, and so on. Considerable advance has been made for the classification and treatment of primary SS in the past few years. This article reviews the recent classification criteria for primary SS and briefly discusses the conventional and novel therapies of the disease.

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

Access this article

Log in via an institution

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. Ferro F, Marcucci E, Orlandi M, Baldini C, Bartoloni-Bocci E (2017) One year in review 2017: primary Sjogren’s syndrome. Clin Exp Rheumatol 35:179–191

    PubMed  Google Scholar 

  2. Patel R, Shahane A (2014) The epidemiology of Sjögren’s syndrome. Clin Epidemiol 6:247–255

    PubMed  PubMed Central  Google Scholar 

  3. Mavragani CP (2017) Mechanisms and new strategies for Sjögren’s syndrome. Annu Rev Med 68:331–343

    Article  PubMed  CAS  Google Scholar 

  4. Both T, Dalm VA, van Hagen PM, van Daele PL (2017) Reviewing primary Sjögren’s syndrome: beyond the dryness—from pathophysiology to diagnosis and treatment. Int J Med Sci 14:191–200

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  5. Verstappen GM, Meiners PM, Corneth OBJ, Visser A, Arends S, Abdulahad WH, Hendriks RW, Vissink A, Kroese FGM, Bootsma H (2017) Abatacept attenuates T follicular helper-cell-dependent B-cell hyperactivity in primary Sjögren’s syndrome. Arthritis Rheumatol 69:1850–1861

    Article  PubMed  CAS  Google Scholar 

  6. Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, Daniels TE, Fox PC, Fox RI, Kassan SS, Pillemer SR, Talal N, Weisman MH, European Study Group on Classification Criteria for Sjögren's Syndrome (2002) Classification criteria for Sjogren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 61:554–558

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  7. Gonzales JA, Lietman TM (2018) Ocular involvement in Sjögren’s syndrome: advances in therapy. Curr Treatm Opt Rheumatol 4:99–109

    Article  Google Scholar 

  8. Nair JJ, Singh TP (2017) Sjogren’s syndrome: review of the aetiology, pathophysiology & potential therapeutic interventions. J Clin Exp Dent 9:584–589

    Google Scholar 

  9. Lojacono A, Nascimbeni G, Favero M et al (2013) Novel aspects of Sjogren’s syndrome in 2012. BMC Med 11:93–110

    Article  PubMed  PubMed Central  Google Scholar 

  10. Shiboski SC, Shiboski CH, Criswell L et al (2012) American College of Rheumatology Classification Criteria for Sjögren’s syndrome: a data-driven, expert consensus approach in the SICCA cohort. Arthritis Care Res 64:475–487

    Article  CAS  Google Scholar 

  11. Sankar V, Noll JL, Brennan MT (2014) Diagnosis of Sjögren’s syndrome: American-European and the American College of Rheumatology Classification Criteria. Atlas Oral Maxillofac Surg Clin North Am 26:13–22

    Article  Google Scholar 

  12. Chu CS, Chi CM (2017) Diagnosis and treatment of primary Sjogren’s syndrome: an update. HK Bull Rheum Dis 17:6–11

    Google Scholar 

  13. Huo AP, Lin KC, Chou CT (2010) Predictive and prognostic value of antinuclear antibodies and rheumatoid factor in primary Sjögren’s syndrome. Int J Rheum Dis 13:39–47

    Article  PubMed  Google Scholar 

  14. Rasmussen A, Ice JA, Li H, Grundahl K, Kelly JA, Radfar L, Stone DU, Hefner KS, Anaya JM, Rohrer M, Gopalakrishnan R, Houston GD, Lewis DM, Chodosh J, Harley JB, Hughes P, Maier-Moore JS, Montgomery CG, Rhodus NL, Farris AD, Segal BM, Jonsson R, Lessard CJ, Scofield RH, Sivils KLM (2014) Comparison of the American-European Consensus Group Sjögren’s syndrome classification criteria to newly proposed American College of Rheumatology criteria in a large, carefully characterized sicca cohort. Ann Rheum Dis 73:31–47

    Article  PubMed  Google Scholar 

  15. Franceschini F, Cavazzana I, Andreoli L, Tincani A (2017) The 2016 classification criteria for primary Sjogren’s syndrome: what’s new. BMC Med 15:69–71

    Article  PubMed  PubMed Central  Google Scholar 

  16. Baer AN, Mcadams DeMarco M, Shiboski SC et al (2015) The SSB-positive/SSA-negative antibody profile is not associated with key phenotypic features of Sjogren’s syndrome. Ann Rheum Dis 74:1557–1561

    Article  PubMed  CAS  Google Scholar 

  17. Billings M, Amin Hadavand M (2018) Comparative analysis of the 2016 ACR-EULAR and the 2002 AECG classification criteria for Sjögren’s syndrome: findings from the B GNIH cohort. Oral Dis 24:184–190

    Article  PubMed  CAS  Google Scholar 

  18. Vitali C, Del PN (2017) Classification and diagnostic criteria in Sjogren’s syndrome: a long-standing and still open controversy. Ann Rheum Dis 76:1953–1954

    Article  PubMed  Google Scholar 

  19. Kuklinski E, Asbell PA (2017) Sjogren’s syndrome from the perspective of ophthalmology. Clin Immunol 182:55–61

    Article  PubMed  CAS  Google Scholar 

  20. Wang K, Shi L, Yu Z, Deng Z, He A, Li S, Liu L (2015) Cyclosporine A suppresses the activation of the Th17 cells in patients with primary Sjogren’s syndrome. Iran J Allergy Asthma Immunol 14:198–207

    PubMed  Google Scholar 

  21. Kawakita T, Shimmura S, Tsubota K (2015) Effect of oral pilocarpine in treating severe dry eye in patients with Sjogren syndrome. Asia Pac J Ophthalmol (Phila) 4:101–105

    Article  CAS  Google Scholar 

  22. Shih KC, Lun CN, Jhanji V, Thong YH, Tong L (2017) Systematic review of randomized controlled trials in the treatment of dry eye disease in Sjogren syndrome. J Inflamm 14:26–37

    Article  Google Scholar 

  23. Petrone D, Condemi JJ, Fife R, Gluck O, Cohen S, Dalgin P (2002) A double-blind, randomized, placebo-controlled study of cevimeline in Sjogren’s syndrome patients with xerostomia and keratoconjunctivitis sicca. Arthritis Rheumatol 46:748–754

    Article  CAS  Google Scholar 

  24. Ono M, Takamura E, Shinozaki K, Tsumura T, Hamano T, Yagi Y, Tsubota K (2004) Therapeutic effect of cevimeline on dry eye in patients with Sjogren’s syndrome: a randomized, double-blind clinical study. Am J Ophthalmol 138:6–17

    Article  PubMed  CAS  Google Scholar 

  25. Demarchi J, Papasidero S, Medina MA, Klajn D, Chaparro del Moral R, Rillo O, Martiré V, Crespo G, Secco A, Catalan Pellet A, Amitrano C, Crow C, Asnal C, Pucci P, Caeiro F, Benzanquen N, Pirola JP, Mayer M, Zazzetti F, Velez S, Barreira J, Tamborenea N, Santiago L, Raiti L (2017) Primary Sjögren’s syndrome: extraglandular manifestations and hydroxychloroquine therapy. Clin Rheumatol 36:2455–2460

    Article  PubMed  CAS  Google Scholar 

  26. Papa ND, Vitali C (2018) Management of primary Sjögren’s syndrome: recent developments and new classification criteria. Ther Adv Musculoskelet Dis 10:39–54

    Article  PubMed  PubMed Central  Google Scholar 

  27. Wang SQ, Zhang LW, Wei P, Hua H (2017) Is hydroxychloroquine effective in treating primary Sjogren’s syndrome: a systematic review and meta-analysis. BMC Musculoskelet Disord 18:186–199

    Article  PubMed  PubMed Central  Google Scholar 

  28. Kedor C, Zernicke J, Hagemann A, Gamboa LM, Callhoff J, Burmester GR, Feist E (2016) A phase II investigator initiated pilot study with low dose cyclosporine A for the treatment of articular involvement in primary Sjogren’s syndrome. Clin Rheumatol 35:2203–2210

    Article  PubMed  Google Scholar 

  29. Cornec D, Jamin C, Pers O (2014) Sjogren’s syndrome: where do we stand, and where shall we go? J Autoimmun 51:109–114

    Article  PubMed  Google Scholar 

  30. Saraux A, Pers JO, Devauchelle-Pensec V (2016) Treatment of primary Sjogren syndrome. Nat Rev Rheumatol 12:456–471

    Article  PubMed  CAS  Google Scholar 

  31. Bowman SJ, Everett CC, O'Dwyer JL et al (2017) Randomized controlled trial of rituximab and cost-effectiveness analysis in treating fatigue and oral dryness in primary Sjogren’s syndrome. Arthritis Rheumatol 43:1440–1450

    Article  CAS  Google Scholar 

  32. Mekinian A, Ravaud P, Hatron PY, Larroche C, Leone J, Gombert B (2012) Efficacy of rituximab in primary Sjogren’s syndrome with peripheral nervous system involvement: results from the AIR registry. Ann Rheum Dis 71:84–87

    Article  PubMed  CAS  Google Scholar 

  33. Seror R, Sordet C, Guillevin L, Hachulla E, Masson C, Ittah M, Candon S, le Guern V, Aouba A, Sibilia J, Gottenberg JE, Mariette X (2007) Tolerance and efficacy of rituximab and changes in serum B cell biomarkers in patients with systemic complications of primary Sjögren’s syndrome. Ann Rheum Dis 66:351–357

    Article  PubMed  CAS  Google Scholar 

  34. Mariette X, Le RS, Quartuccio L et al (2015) Efficacy and safety of belimumab in primary Sjogren's syndrome: results of the BELISS open-label phase II study. Ann Rheum Dis 74:526–531

    Article  PubMed  CAS  Google Scholar 

  35. Quartuccio L, Salvin S, Corazza L, Gandolfo S, Fabris M, De VS (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 

  36. Mavragani CP (2017) Mechanisms and new strategies for primary Sjögren’s syndrome. Annu Rev Med 68:331–343

    Article  PubMed  CAS  Google Scholar 

  37. Sieger N, Fleischer SJ, Mei HE, Reiter K, Shock A, Burmester GR, Daridon C, Dörner T (2013) CD22 ligation inhibits downstream B cell receptor signaling and Ca(2+), flux upon activation. Arthritis Rheum 65:770–779

    Article  PubMed  CAS  Google Scholar 

  38. Steinfeld SD, Tant L, Burmester GR, Teoh NKW, Wegener WA, Goldenberg DM, Pradier O (2006) Epratuzumab (humanised anti-CD22 antibody) in primary Sjogren’s syndrome: an open-label phase I/II study. Arthritis Res Ther 8:R129

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  39. Tsuboi H, Matsumoto I, Hagiwara S, Hirota T, Takahashi H, Ebe H, Yokosawa M, Hagiya C, Asashima H, Takai C, Miki H, Umeda N, Kondo Y, Ogishima H, Suzuki T, Hirata S, Saito K, Tanaka Y, Horai Y, Nakamura H, Kawakami A, Sumida T (2015) Efficacy and safety of abatacept for patients with Sjögren’s syndrome associated with rheumatoid arthritis: rheumatoid arthritis with orencia trial toward Sjögren’s syndrome Endocrinopathy (ROSE) trial-an open-label, one-year, prospective study. Interim ana. Mod Rheumatol 25:187–193

    Article  PubMed  CAS  Google Scholar 

  40. Nocturne G, Cornec D, Seror R, Mariette X (2016) Use of biologics in Sjögren’s syndrome. Rheum Dis Clin N Am 42:407–417

    Article  Google Scholar 

  41. Mariette X, Ravaud P, Steinfeld S et al (2014) Inefficacy of infliximab in primary Sjögren’s syndrome: results of the randomized, controlled trial of remicade in primary Sjögren’s syndrome (TRIPSS). Arthritis Rheumatol 50:1270

    Article  CAS  Google Scholar 

  42. Türkoğlu EB, Tuna S, Alan S et al (2015) Effect of systemic infliximab therapy in patients with Sjögren’s syndrome/Sekonder Sjogren sendromlu hastalarda sistemik infliksimab tedavisinin etkinligi. Turk J Ophthalmol 45:138–141

    Article  Google Scholar 

  43. Nakahara H, Kawamoto K, Mori H et al (2014) AB0485 tocilizumab is effective for the patient with Sjogren’s syndrome complicated with rheumatoid arthritis. Ann Rheum Dis 73:967–968

    Google Scholar 

  44. Marino A, Narula S, Lerman M (2017) First pediatric patient with Neuromyelitis optica and Sjogren’s syndrome successfully treated with tocilizumab. Pediatr Neurol 73:5–6

    Article  Google Scholar 

  45. Wei W (2016) Soft regulation of inflammatory immune response. CPB 32:297–303

    Google Scholar 

Download references

Acknowledgements

Key projects of National natural science foundation (no. 81330081).

Key projects of Anhui province university outstanding youth talent fund (gxyqZD2018023).

The Anhui Province Nature Science Foundation in the University (no. kj2018a0166).

Author information

Authors and Affiliations

  1. Institute of Clinical Pharmacology, Anhui Medical University, Hefei,, 230032, Anhui, China

    Xiaoyun Chen, Huaxun Wu & Wei Wei

  2. Key Laboratory of Anti-inflammatory and Immune Medicine, Ministry of Education, Hefei, 230032, Anhui, China

    Xiaoyun Chen, Huaxun Wu & Wei Wei

  3. Anhui Collaborative Innovation Center of Anti-inflammatory and Immune Medicine, Hefei, 230032, Anhui, China

    Xiaoyun Chen, Huaxun Wu & Wei Wei

Authors
  1. Xiaoyun Chen
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Huaxun Wu
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Wei Wei
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding authors

Correspondence to Huaxun Wu or Wei Wei.

Ethics declarations

Conflict of interest statement

We declare that we have no financial and personal relationships with other people or organizations that can inappropriately influence our work, there is no professional or other personal interest of any nature or kind in any product, service and/or company that could be construed as influencing the position presented in, or the review of, the manuscript entitled, “Advances in the diagnosis and treatment of Sjogren’s syndrome.”

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Chen, X., Wu, H. & Wei, W. Advances in the diagnosis and treatment of Sjogren’s syndrome. Clin Rheumatol 37, 1743–1749 (2018). https://doi.org/10.1007/s10067-018-4153-8

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-018-4153-8

Keywords

Search

Navigation