Early onset primary Sjögren syndrome, clinical and laboratory characteristics



Primary Sjögren syndrome (pSS) is usually encountered between the fourth and sixth decades. It is known that the age of onset in autoimmune diseases may affect the clinical features. In this study, we aimed to investigate the clinical and laboratory characteristics of early onset pSS patients.


The data of 352 pSS patients were analyzed retrospectively. The patients were divided into two groups as those with the onset age of 35 or younger (early-onset) and those with the onset age of older than 35. The clinical, laboratory, and serological characteristics of the two groups were compared. p < 0.05 was considered statistically significant.


Forty patients in the group with an onset age of 35 or younger (11.4%) and 312 patients with an onset age of older than 35 (88.6%) were analyzed. The frequency of skin (22.5% vs 1.9%, p < 0.001) and renal involvement (10% vs 2.2%, p = 0.026) was significantly higher in the early-onset group than the late-onset group. There was no significant difference between the two groups in terms of xerostomia, eye dryness, arthritis, and other systemic involvement. Anti-Ro52 positivity (p = 0.04), elevated serum IgG levels (p = 0.004), and low C4 (p = 0.002) presence were more frequent in the early-onset group.


Consequently, it is seen that the clinical phenotype of early-onset pSS patients may be different to those with later onset. Especially the more frequent observation of poor prognostic factors at early-onset ages shows the necessity to monitor these patients more regularly.

Key points:
• The clinical and laboratory features of patients with early-onset primary Sjogren syndrome may differ from late-onset patients.

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  1. 1.

    Mavragani CP, Moutsopoulos HM (2010) The geoepidemiology of Sjögren’s syndrome. Autoimmun Rev 9(5):A305–A310

    Article  Google Scholar 

  2. 2.

    Jülich M, Kanne A-M, Sehnert B, Budweiser S, Voll RE, Kollert F (2018) Serological lymphocytic activity and patient-reported outcomes in Sjögren’s syndrome. Clin Rheumatol 37(9):2361–2366

    Article  Google Scholar 

  3. 3.

    Singh AG, Singh S, Matteson EL (2015) Rate, risk factors and causes of mortality in patients with Sjögren’s syndrome: a systematic review and meta-analysis of cohort studies. Rheumatology 55(3):450–460

    PubMed  Google Scholar 

  4. 4.

    Ramos-Casals M, Font J (2005) Primary Sjogren’s syndrome: current and emergent aetiopathogenic concepts. Rheumatology 44(11):1354–1367

    CAS  Article  Google Scholar 

  5. 5.

    Helmick CG, Felson DT, Lawrence RC, Gabriel S, Hirsch R, Kwoh CK, Liang MH, Kremers HM, Mayes MD, Merkel PA (2008) Estimates of the prevalence of arthritis and other rheumatic conditions in the United States: part I. Arthritis Rheum 58(1):15–25

    Article  Google Scholar 

  6. 6.

    Kassan SS, Moutsopoulos HM (2004) Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med 164(12):1275–1284

    Article  Google Scholar 

  7. 7.

    Amador-Patarroyo MJ, Rodriguez-Rodriguez A, Montoya-Ortiz G (2012) How does age at onset influence the outcome of autoimmune diseases? Autoimmune Dis 2012

  8. 8.

    Ramos-Casals M, Cervera R, Font J, Garcóa-Carrasco M, Espinosa G, Reino S, Pallares L, Ingelmo M (1998) Young onset of primary Sjögren’s syndrome: clinical and immunological characteristics. Lupus 7(3):202–206

    CAS  Article  Google Scholar 

  9. 9.

    Garcia-Carrasco M, Ramos-Casals M, Rosas J, Pallares L, Calvo-Alen J, Cervera R, Font J, Ingelmo M (2002) Primary Sjögren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine 81(4):270–280

    Article  Google Scholar 

  10. 10.

    Ramos-Casals M, Solans R, Rosas J, Camps MT, Gil A, del Pino-Montes J, Calvo-Alen J, Jiménez-Alonso J, Micó M-L, Beltrán J (2008) Primary Sjögren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine 87(4):210–219

    CAS  Article  Google Scholar 

  11. 11.

    Tishler M, Yaron I, Shirazi I, Yaron M (2001) Clinical and immunological characteristics of elderly onset Sjögren’s syndrome: a comparison with younger onset disease. J Rheumatol 28(4):795–797

    CAS  PubMed  Google Scholar 

  12. 12.

    Botsios C, Furlan A, Ostuni P, Sfriso P, Andretta M, Ometto F, Raffeiner B, Todesco S, Punzi L (2011) Elderly onset of primary Sjögren’s syndrome: clinical manifestations, serological features and oral/ocular diagnostic tests. Comparison with adult and young onset of the disease in a cohort of 336 Italian patients. Joint Bone Spine 78(2):171–174

    Article  Google Scholar 

  13. 13.

    Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, Rasmussen A, Scofield H, Vitali C, Bowman SJ (2017) 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren’s syndrome: a consensus and data-driven methodology involving three international patient cohorts. Arthritis Rheumatol 69(1):35–45

    Article  Google Scholar 

  14. 14.

    Aljohani R, Gladman D, Su J, Urowitz M (2017) Disease evolution in late-onset and early-onset systemic lupus erythematosus. Lupus 26(11):1190–1196

    CAS  Article  Google Scholar 

  15. 15.

    Tucker L, Uribe A, Fernandez M, Vila L, McGwin G, Apte M, Fessler B, Bastian H, Reveille J, Alarcón G (2008) Adolescent onset of lupus results in more aggressive disease and worse outcomes: results of a nested matched case–control study within LUMINA, a multiethnic US cohort (LUMINA LVII). Lupus 17(4):314–322

    CAS  Article  Google Scholar 

  16. 16.

    Carreira PE, Carmona L, Joven B, Loza E, Andréu J, Riemekasten G, Vettori S, Balbir-Gurman A, Airò P, Walker U (2019) Differences associated with age at onset in early systemic sclerosis patients: a report from the EULAR Scleroderma Trials and Research Group (EUSTAR) database. Scand J Rheumatol 48(1):42–51

    CAS  Article  Google Scholar 

  17. 17.

    Anquetil C, Hachulla E, Machuron F, Mariette X, Le Guern V, Vittecoq O, Dernis E, Larroche C, Dubost J-J, Perdriger A (2019) Is early-onset primary Sjögren’s syndrome a worse prognosis form of the disease? Rheumatology 58(7):1163–1167

    Article  Google Scholar 

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The authors would like to thank Dr. Ufuk İlgen for his contribution to statistical work.

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Correspondence to Müçteba Enes Yayla.

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Yayla, M.E., Karaman, Z., Torgutalp, M. et al. Early onset primary Sjögren syndrome, clinical and laboratory characteristics. Clin Rheumatol 39, 2689–2696 (2020). https://doi.org/10.1007/s10067-020-05017-3

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  • Early-onset
  • Epidemiology
  • Sjogren’s syndrome