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From syndrome to disease: the evolution of the nomenclature of Sjögren’s disease

https://doi.org/10.14412/1996-7012-2025-6-7-12

Abstract

The article discusses current concepts of Sjögren’s disease as an independent multisystem autoimmune disorder with a wide spectrum of organ manifestations and a high risk of lymphoproliferative complications. Special attention is given to new data on pathogenetic mechanisms based on B-cell hyperactivity. The most advanced approaches to monitoring and treatment are discussed, including the implementation of activity indices and the prospects of targeted therapy.

About the Authors

A. L. Maslyansky
Almazov National Medical Research Centre, Ministry of Health of Russia; V.A. Nasonova Research Institute of Rheumatology; Saint Petersburg State University
Russian Federation

2, Akkuratova Street, Saint Petersburg 197341; 34A, Kashirskoye Shosse, Moscow 1155224 7–9, Universitetskaya Embankment, Saint Petersburg 199034



A. V. Torgashina
V.A. Nasonova Research Institute of Rheumatology
Russian Federation

Anna Vasilievna Torgashina

34A, Kashirskoye Shosse, Moscow 115522



References

1. Jonsson R. Henrik Sjögren (1899-1986): the syndrome and his legacy. Ann Rheum Dis. 2021 Sep;80(9):1108-1109. doi: 10.1136/annrheumdis-2021-219942.

2. Moutsopoulos HM, Mann DL, Johnson AH, Chused TM. Genetic differences between primary and secondary sicca syndrome. N Engl J Med. 1979 Oct 4;301(14):761-3. doi: 10.1056/NEJM197910043011405.

3. Björk A, Mofors J, Wahren-Herlenius M. Environmental factors in the pathogenesis of primary Sjögren's syndrome. J Intern Med. 2020 May;287(5):475-492. doi: 10.1111/joim.13032.

4. Beydon M, McCoy S, Nguyen Y, et al. Epidemiology of Sjögren syndrome. Nat Rev Rheumatol. 2024 Mar;20(3):158-169. doi: 10.1038/s41584-023-01057-6.

5. Baer AN, Hammitt KM. Sjögren’s disease, not syndrome. Arthritis Rheumatol. 2021 Jul; 73(7):1347-1348. doi: 10.1002/art.41676.

6. Ramos-Casals M, Baer AN, Brito-Zerуn MDP, et al. 2023 International Rome consensus for the nomenclature of Sjögren disease. Nat Rev Rheumatol. 2025 Jul;21(7): 426-437. doi: 10.1038/s41584-025-01268-z.

7. Mavragani CP, Moutsopoulos HM. Primary versus secondary Sjögren syndrome: is it time to reconsider these terms? J Rheumatol. 2019 Jul;46(7):665-666. doi: 10.3899/jrheum.180392.

8. Baldini C, Bartoloni E, Bombardieri M. Highlights of the 15th international symposium on Sjögren’s syndrome. Clin Exp Rheumatol. 2022 Dec;40(12):2201-2210. doi: 10.55563/clinexprheumatol/yt6g9t.

9. Baldini C, Chatzis LG, Fulvio G, et al. Pathogenesis of Sjögren's disease: one year in review 2024. Clin Exp Rheumatol. 2024 Dec; 42(12):2336-2343. DOI: 10.55563/clinex-prheumatol/i8iszc.

10. Liu Z, Chu A. Sjögren’s Syndrome and Viral Infections. Rheumatol Ther. 2021 Sep;8(3): 1051-1059. doi: 10.1007/s40744-021-00334-8.

11. Li L, Shen S, Shao S, et al. The role of B cell-activating factor system in autoimmune diseases: mechanisms, disease implications, and therapeutic advances. Front Immunol. 2025 Jun 6:16:1538555. doi: 10.3389/fimmu.2025.1538555.

12. Ponomarev AB, Pashaeva KR, Feizieva NYu. Comparative analysis of the mechanisms of development of cryoglobulinemic vasculitis and Sjögren's syndrome. Arkhiv patologii. 2024;86(1):52 56. (In Russ.).

13. Verstappen GM, Kroese FGM, Bootsma H, et al. T cells in primary Sjögren's syndrome: targets for early intervention.Rheumatology(Oxford). 2021 Jul 1;60(7):3088 3098. doi: 10.1093/rheumatology/kez004.

14. Lessard C J, Li H, Adrianto I, et al. Variants at multiple loci implicated in both innate and adaptive immune responses are associated with Sjögren’s syndrome. Nat Genet. 2013 Nov; 45(11):1284-1292. doi: 10.1038/ng.2792.

15. Khatri B, Tessneer KL, Rasmussen A, et al. Genome-wide association study identifies Sjögren’s risk loci with functional implications in immune and glandular cells. Nat Commun. 2022 Jul 26;13(1):4287. doi: 10.1038/s41467-022-30773-y.

16. Thorlacius GE, Hultin-Rosenberg L, Sandling JK, et al. Genetic and clinical basis for two distinct subtypes of primary Sjögren’s syndrome. Rheumatology (Oxford). 2021 Feb 1; 60(2):837-848. doi: 10.1093/rheumatology/keaa331.

17. Thorlacius GE, Bjork A, Wahren-Herlenius M. Genetics and epigenetics of primary Sjögren syndrome: implications for future therapies. Nat Rev Rheumatol. 2023 May; 19(5):288-306. doi: 10.1038/s41584-023-00932-6.

18. Bootsma H, Kroese FGM. Association of Circulating Antibody-Secreting Cell Maturity With Disease Features in Primary Sjögren’s Syndrome. Arthritis Rheumatol. 2023 Jun; 75(6):973-983. doi: 10.1002/art.42422.

19. Baldini C, Fulvio G, La Rocca G, Ferro F. Update on the pathophysiology and treatment of primary Sjögren syndrome. Nat Rev Rheumatol. 2024 Aug;20(8):473-491. doi: 10.1038/s41584-024-01135-3.

20. Meudec L, Debray MP, Beurnier A, et al. Pulmonary involvement in Sjögren syndrome. RMD Open. 2024 Feb 29;10(1): e003866. doi: 10.1136/rmdopen-2023003866.

21. Madej M, Proc K, Wawryka P, et al. The analysis of the pulmonary domain involvement in Sjögren's disease. Ther Adv Musculoskelet Dis. 2024 Dec 16:16:1759720 X241305218. doi: 10.1177/1759720X24 1305218.

22. Chal'tsev BD, Vasil'ev VI, Avetisov IO, et al. Damage to the respiratory tract in Sjögren's disease, focus on interstitial lung disease. Nauchno-prakticheskaya revmatologiya. 2022;60(2):214-227. (In Russ.).

23. Aiyegbusi O, McGregor L, McGeoch L, et al. Renal Disease in Primary Sjögren’s Syndrome. Rheumatol Ther. 2020 Dec 24;8(1): 63–80. doi: 10.1007/s40744-020-00264-x.

24. Porto Fuentes У. Renal involvement in primary Sjögren's syndrome: Beyond tubulointerstitial nephritis. Nefrologia (Engl Ed). 2025 Aug-Sep;45(7):501341. doi: 10.1016/j.nefroe.2025.501341.

25. Barsottin OGP, de Moraes MPM, Fraiman PHA, et al. Sjögren's syndrome: a neurological perspective. Arq Neuropsiquiatr. 2023 Dec 29;81(12):1077–1083. doi: 10.1055/s-0043-1777105.

26. Breillat P, Le Guern V, d'Humieres T, et al. Cutaneous Vasculitis in Primary Sjögren Disease. JAMA Dermatol. 2025 Aug 6;161(10): 1057-62. doi: 10.1001/jamadermatol.2025.2665.

27. Torgashina AV. A modern view on the treatment of Sjögren's disease. Farmateka. 2020; 27(7):72 80. (In Russ.).

28. Vasil'ev MI, Probatova NA, Tupitsyn NN, et al. Lymphoproliferative diseases in Sjögren's disease. Onkogematologiya. 2007;(3):16-26. (In Russ.).

29. Rusinovich-Lovgach O, Plaza Z, Fernandez-Castro M, el al. High incidence of lymphoma in Sjögren's disease: predictors and mortality implications in a prospective cohort study. Rheumatol Int. 2025 Aug 6;45(8):184. doi: 10.1007/s00296-025-05935-y.

30. Shetler K. Management of Gastrointestinal Manifestations in Sjцgren’s. In: Wallace DJ, editor. The Sjögren's Book 5. Oxford: Oxford University Press; 2022. P. 331–337.

31. Brito-Zerуn P, Kostov B, Solans R, et al. Systemic activity and mortality in primary Sjögren syndrome: Predicting survival using the EULAR-SS Disease Activity Index (ESSDAI) in 1045 patients. Ann Rheum Dis. 2016 Feb;75(2):348-55. doi: 10.1136/annrheumdis-2014-206418.

32. Duarte C, Ferreira RJO, Santos EJF, da Silva JAP. Treating-to-target in rheumatology: Theory and practice. Best Pract Res Clin Rheumatol. 2022 Mar;36(1):101735. doi: 10.1016/j.berh.2021.101735.

33. Brito-Zerуn P, Ramos-Casals M, Benga G, et al. Efficacy and safety of topical and systemic medications: a systematic literature review informing the EULAR recommendations for the management of Sjögren’s syndrome. RMD Open. 2019 Oct 28;5(2):e001064. doi: 10.1136/rmdopen-2019-001064.

34. Seror R, Ravaud P, Bowman SJ, et al. EULAR Sjogren’s syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjögren’s syndrome. Ann Rheum Dis. 2010 Jun;69(6): 1103-9. doi: 10.1136/ard.2009.110619.

35. Seror R, Bowman SJ, Brito-Zeron P, et al. EULAR Sjögren's syndrome disease activity index (ESSDAI): a user guide. RMD Open. 2015 Feb 20;1(1):e000022. doi: 10.1136/rmdopen-2014-000022.

36. Ture HY, Kim NR, Nam EJ. EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) and Other Patient-Reported Outcomes in the Assessment of Glandular Dysfunction in Primary Sjögren's Syndrome. Life (Basel). 2023 Sep 29;13(10):1991. doi: 10.3390/life13101991.

37. Isenberg DA. CRESS: improving the assessment of disease activity in Sjögren's syndrome. Lancet Rheumatol. 2021 Aug;3(8): e534-e535. doi: 10.1016/S2665-9913(21)00150-8.

38. Fox PC, Datiles M, Atkinson JC, Macynski AA, et al. Prednisone and piroxicam for treatment of primary Sjögren's syndrome. Clin Exp Rheumatol. 1993 Mar-Apr;11(2):149-156.

39. Gottenberg JE, Ravaud P, Puechal X, et al. Effects of hydroxychloroquine on symptomatic improvement in primary Sjögren syndrome the JOQUER randomized clinical trial. JAMA. 2014 Jul 16;312(3):249-58. doi: 10.1001/jama.2014.7682.

40. Skopouli FN, Jagiello P, Tsifetaki N, Moutsopoulos HM. Methotrexate in primary Sjögren’s syndrome. Clin Exp Rheumatol. 1996 Sep-Oct;14(5):555-558.

41. Price E, Rigby S, Clancy U, Venables PA. Double blind placebo controlled trial of azathioprine in the treatment of primary Sjögren’s syndrome. J Rheumatol. 1998 May; 25(5):896-9.

42. Felten R, Devauchelle-Pensec V, Seror R, et al. Interleukin 6 receptor inhibition in primary Sjögren syndrome: a multicentre double-blind randomised placebo-controlled trial. Ann Rheum Dis. 2021 Mar;80(3):329-338. doi: 10.1136/annrheumdis-2020-218467.

43. Mariette X, Ravaud P, Steinfeld S, et al. Inefficacy of infliximab in primary Sjögren's syndrome: results of the randomized, controlled Trial of Remicade in Primary Sjögren's Syndrome (TRIPSS). Arthritis Rheum. 2004 Apr;50(4):1270-6. doi: 10.1002/art.20146.

44. Baer AN, Gottenberg JE, E St Clair W, et al. Efficacy and safety of abatacept in active primary Sjögren's syndrome: results of a phase III, randomised, placebo-controlled trial. Ann Rheum Dis. 2021;80(3):339-348. doi: 10.1136/annrheumdis-2020-218599.

45. Meletis J, Gottenberg JE, Mariette X, et al. Efficacy of Rituximab in Primary Sjögren’s Syndrome: The TEARS Randomized, Placebo-Controlled Trial. Ann Intern Med. 2014 Feb 18;160(4):233-42. doi: 10.7326/M13-1085.

46. Ritter J, Chen Y, Stefansk AL, Dörner T. Current and future treatment in primary Sjögren's syndrome – A still challenging development. Joint Bone Spine. 2022 Nov;89(6): 105406. doi: 10.1016/j.jbspin.2022.105406.

47. De Vita S, Quartuccio L, Seror R, et al. Efficacy and safety of belimumab given for 12 months in primary Sjögren's syndrome: the BELISS open-label phase II study. Rheumatology (Oxford). 2015 Dec;54(12):2249-56. doi: 10.1093/rheumatology/kev257.

48. Mariette X, Barone F, Baldini C, et al. A randomized, phase II study of sequential belimumab and rituximab in primary Sjögren's syndrome. JCI Insight. 2022 Dec 8;7(23): e163030. doi: 10.1172/jci.insight.163030.

49. Dörner T, Posch MG, Li Y, et al. Treatment of primary Sjögren's syndrome with ianalumab, an anti-BAFF receptor monoclonal antibody. Ann Rheum Dis. 2019 May; 78(5):641-647. doi: 10.1136/annrheumdis-2018-214720.

50. Bowman SJ, Everett CC, O'Dwyer JL, et al. Safety and efficacy of subcutaneous ianalumab (VAY736) in patients with primary Sjögren's syndrome: a randomised, double-blind, placebo-controlled, phase 2b dose-finding trial. Lancet. 2022 Jan 8;399(10320):161-171. doi: 10.1016/S0140-6736(21)02251-0.

51. Dörner T, Bowman SJ, Fox R, et al. Safety and Efficacy of Ianalumab in Patients With Sjögren's Disease: 52-Week Results From a Randomized, Placebo-Controlled, Phase 2b Dose-Ranging Study. Arthritis Rheumatol. 2025 May;77(5):560-570. doi: 10.1002/art.43059.

52. Gottenberg JE, Sivils K, Campbell K, et al. Efficacy and Safety of nipocalimab, an antiFcRn monoclonal antibody, in primary Sjögren’s disease: Results from a phase 2, multicenter, randomized, placebo-controlled, double-blind study (DAHLIAS). Ann Rheum Dis. 2024;83(1):240.


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For citations:


Maslyansky AL, Torgashina AV. From syndrome to disease: the evolution of the nomenclature of Sjögren’s disease. Sovremennaya Revmatologiya=Modern Rheumatology Journal. 2025;19(6):7-12. (In Russ.) https://doi.org/10.14412/1996-7012-2025-6-7-12

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ISSN 1996-7012 (Print)
ISSN 2310-158X (Online)