Skip to main content
Top
Published in: Clinical Rheumatology 9/2020

01-09-2020 | Original Article

Early onset primary Sjögren syndrome, clinical and laboratory characteristics

Authors: Müçteba Enes Yayla, Zehra Karaman, Murat Torgutalp, Ayşe Bahar Keleşoğlu Dinçer, Emine Gözde Aydemir Gülöksüz, Serdar Sezer, Didem Şahin Eroğlu, Mehmet Levent Yüksel, Tahsin Murat Turgay, Gülay Kınıklı, Aşkın Ateş

Published in: Clinical Rheumatology | Issue 9/2020

Login to get access

Abstract

Introduction/objectives

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.

Method

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.

Results

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.

Conclusions

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.
Literature
1.
go back to reference Mavragani CP, Moutsopoulos HM (2010) The geoepidemiology of Sjögren’s syndrome. Autoimmun Rev 9(5):A305–A310CrossRef Mavragani CP, Moutsopoulos HM (2010) The geoepidemiology of Sjögren’s syndrome. Autoimmun Rev 9(5):A305–A310CrossRef
2.
go back to reference 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):2361–2366CrossRef 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):2361–2366CrossRef
3.
go back to reference 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):450–460PubMed 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):450–460PubMed
4.
go back to reference Ramos-Casals M, Font J (2005) Primary Sjogren’s syndrome: current and emergent aetiopathogenic concepts. Rheumatology 44(11):1354–1367CrossRef Ramos-Casals M, Font J (2005) Primary Sjogren’s syndrome: current and emergent aetiopathogenic concepts. Rheumatology 44(11):1354–1367CrossRef
5.
go back to reference 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):15–25CrossRef 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):15–25CrossRef
6.
go back to reference Kassan SS, Moutsopoulos HM (2004) Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med 164(12):1275–1284CrossRef Kassan SS, Moutsopoulos HM (2004) Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med 164(12):1275–1284CrossRef
7.
go back to reference Amador-Patarroyo MJ, Rodriguez-Rodriguez A, Montoya-Ortiz G (2012) How does age at onset influence the outcome of autoimmune diseases? Autoimmune Dis 2012 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.
go back to reference 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):202–206CrossRef 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):202–206CrossRef
9.
go back to reference 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):270–280CrossRef 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):270–280CrossRef
10.
go back to reference 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):210–219CrossRef 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):210–219CrossRef
11.
go back to reference 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):795–797PubMed 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):795–797PubMed
12.
go back to reference 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):171–174CrossRef 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):171–174CrossRef
13.
go back to reference 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):35–45CrossRef 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):35–45CrossRef
14.
go back to reference Aljohani R, Gladman D, Su J, Urowitz M (2017) Disease evolution in late-onset and early-onset systemic lupus erythematosus. Lupus 26(11):1190–1196CrossRef Aljohani R, Gladman D, Su J, Urowitz M (2017) Disease evolution in late-onset and early-onset systemic lupus erythematosus. Lupus 26(11):1190–1196CrossRef
15.
go back to reference 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):314–322CrossRef 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):314–322CrossRef
16.
go back to reference 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):42–51CrossRef 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):42–51CrossRef
17.
go back to reference 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):1163–1167CrossRef 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):1163–1167CrossRef
Metadata
Title
Early onset primary Sjögren syndrome, clinical and laboratory characteristics
Authors
Müçteba Enes Yayla
Zehra Karaman
Murat Torgutalp
Ayşe Bahar Keleşoğlu Dinçer
Emine Gözde Aydemir Gülöksüz
Serdar Sezer
Didem Şahin Eroğlu
Mehmet Levent Yüksel
Tahsin Murat Turgay
Gülay Kınıklı
Aşkın Ateş
Publication date
01-09-2020
Publisher
Springer International Publishing
Published in
Clinical Rheumatology / Issue 9/2020
Print ISSN: 0770-3198
Electronic ISSN: 1434-9949
DOI
https://doi.org/10.1007/s10067-020-05017-3

Other articles of this Issue 9/2020

Clinical Rheumatology 9/2020 Go to the issue