This study was conducted to determine long-term survival rates and the factors associated with mortality in Turkish primary Sjögren syndrome (pSS) patients.
All patients diagnosed with pSS between 2004 and 2014 were included in this study. By January 2019, all subjects still living by the end of the study, as well as any death, were identified. Survival rates and standard mortality rates (SMRs) using general population mortality data were calculated. Mortality-related factors were determined by univariate and multivariate analysis.
During follow-up, 33 cases of 372 pSS patients resulted in death (8.9%). Of those patients, they were typically older at disease onset, at recruitment, and had shorter follow-up times (p < 0.001 for all). The overall SMR of all pSS patients compared with the general population was 2.11 (95% confidence interval (CI) 1.39–2.83). Male pSS patients had a higher SMR than that of general male patients. Overall survival rates were 97.8% at five years, 90.2% at 10 years, and 87.1% at 15 years in patients with pSS. The survival rate of pSS patients was significantly lower than the general Turkish population (p = 0.011). Multivariate Cox regression analysis showed that older age at disease onset and the presence of interstitial lung disease (ILD) were independent risk factors for mortality.
Based on these data, mortality rates of Turkish pSS patients are higher compared with the general population. Survival significantly decreased in the pSS patients with ILD, especially in older male patients at disease onset. Male gender and malignancy may also be associated with a worse prognosis in pSS patients.
• Mortality in Sjögren’s syndrome.
This is a preview of subscription content, log in to check access.
Buy single article
Instant unlimited access to the full article PDF.
Price includes VAT for USA
Subscribe to journal
Immediate online access to all issues from 2019. Subscription will auto renew annually.
This is the net price. Taxes to be calculated in checkout.
Fox RI (2005) Sjogren’s syndrome. Lancet 366(9482):321–331
Kassan SS, Moutsopoulos HM (2004) Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med 164:1275–1284
Martens PB, Pillemer SR, Jacobsson LT, O’Fallon WM, Matteson EL (1999) Survivorship in a population based cohort of patients with Sjögren’s syndrome, 1976–1992. J Rheumatol 26:1296–1300
Theander E, Manthorpe R, Jacobsson LT (2004) Mortality and causes of death in primary Sjögren’s syndrome: a prospective cohort study. Arthritis Rheum 50(4):1262–1269
Kruize AA, Hené RJ, van der Heide A, Bodeutsch C, de Wilde PC, van Bijsterveld OP, de Jong J, Feltkamp TE, Kater L, Bijlsma JW (1996) Long-term followup of patients. Arthritis Rheum 39:297–303
Skopouli FN, Dafni U, Ioannidis JP, Moutsopoulos HM (2000) Clinical evolution, and morbidity and mortality of primary Sjögren’s syndrome. Semin Arthritis Rheum. 29(5):296–304
Ioannidis JP, Vassiliou VA, Moutsopoulos HM (2002) Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjögren’s syndrome. Arthritis Rheum 46:741–747
Weng MY, Huang YT, Liu MF, Lu TH (2011) Incidence and mortality of treated primary Sjogren’s syndrome in Taiwan: a population-based study. J Rheumatol 38(4):706–708. https://doi.org/10.3899/jrheum.100883
Moutsopoulos HM (2014) Sjögren’s syndrome: a forty-year scientific journey. J Autoimmun 51:1–9. https://doi.org/10.1016/j.jaut.2014.01.001
Palm Q, Garen T, Enger TB, Jensen JL, Lund MB, Aalokken TM, Gran JT (2013) Clinical pulmonary involvement in primary Sjogren’s syndrome: prevalence, quality of life and mortality-a retrospective study based on registry data. Rheumatology (Oxford) 52(1):173–179. https://doi.org/10.1093/rheumatology/kes311
Voulgarelis M, Tzioufas AG, Moutsopoulos HM (2008) Mortality in Sjögren’s syndrome. Clin Exp Rheumatol 26(5 Suppl 51):S66–S71
Davidson BK, Kelly CA, Griffiths ID (1999) Primary Sjögren’s syndrome in the North East of England: a long-term follow-up study. Rheumatology (Oxford) 38:245–243
Pertovaara M, Pukkala E, Laippala P, Miettinen A, Pasternack A (2001) A longitudinal cohort study of Finnish patients with primary Sjögren’s syndrome: clinical, immunological, and epidemiological aspects. Ann Rheum Dis 60:467–472
Alamanos Y, Tsifetaki N, Voulgari PV, Venetsanopoulou AI, Siozos C, Drosos AA (2006) Epidemiology of primary Sjögren’s syndrome in north-west Greece, 1982–2003. Rheumatology (Oxford) 45:187–191
Brito-Zerón P, Kostov B, Solans R, Fraile G, Suárez-Cuervo C, Casanovas A, Rascón FJ, Qanneta R, Pérez-Alvarez R, Ripoll M, Akasbi M, Pinilla B, Bosch JA, Nava-Mateos J, Díaz-López B, Morera-Morales ML, Gheitasi H, Retamozo S, Ramos-Casals M, SS Study Group, Autoimmune Diseases Study Group (GEAS), Spanish Society of Internal Medicine (SEMI) (2016) Systemic activity and mortality in primary Sjögren syndrome: predicting survival using the EULAR-SS Disease Activity Index (ESSDAI) in 1045 patients. Ann Rheum Dis 75(2):348–355. https://doi.org/10.1136/annrheumdis-2014-206418
Zhang W, Feng S, Yan S, Zhao Y, Li M, Sun J, Zhang FC, Cui Q, Dong Y (2010) Incidence of malignancy in primary Sjogren’s syndrome in a Chinese cohort. Rheumatology (Oxford) 49:571–577
Panchovska M, Sheitanov Y, Uzunov N (2004) Mortality of Bulgarian patients with primary and secondary Sjogren’s syndrome. Bratisl Lek Listy 105(12):434
Kim HJ, Kim KH, Hann HJ, Han S, Kim Y, Lee SH, Kim DS, Ahn HS (2017) Incidence, mortality, and causes of death in physician-diagnosed primary Sjögren’s syndrome in Korea: a nationwide, population-based study. Semin Arthritis Rheum 47(2):222–227. https://doi.org/10.1016/j.semarthrit.2017.03.004
Kabasakal Y, Kitapçıoğlu G, Karabulut G, Tezcan M, Balkarlı A, Aksoy A, Yavuz Ş, Yılmaz S, Kaşifoğlu T, Kalyoncu U, Dalkılıç E, Tufan A, Mercan R, Yıldız F, Şentürk T, Önen F, Bes C, Erken E, Tunç E, Kamalı S, Tarhan E, Yazıcı A, Düzgün N, Bıçakçıgil M, Yılmaz S, Özmen M, Öcal L, Alibaz-Öner F, Solmaz D, Çobankara V, Nalbant S, Kasapoğlu Günal E, Kaşkari D, Göker B (2017) Criteria sets for primary Sjogren’s syndrome are not adequate for those presenting with extraglandular organ involvements as their dominant clinical features. Rheumatol Int 37(5):675–684. https://doi.org/10.1007/s00296-017-3691-8
Shiboski SC, Shiboski CH, Criswell LA, Baer AN, Challacombe S, Lanfranchi H, Schiødt M, Umehara H, Vivino F, Zhao Y, Dong Y, Greenspan D, Heidenreich AM, Helin P, Kirkham B, Kitagawa K, Larkin G, Li M, Lietman T, Lindegaard J, McNamara N, Sack K, Shirlaw P, Sugai S, Vollenweider C, Whitcher J, Wu A, Zhang S, Zhang W, Greenspan JS, Daniels TE, Sjögren’s International Collaborative Clinical Alliance (SICCA) Research Groups (2012) American College of Rheumatology classification criteria for Sjögren’s syndrome: a data-driven, expert consensus approach in the Sjögren’s International Collaborative Clinical Alliance Cohort. Arthritis Care Res 64(4):475–487
Daniels TE, Cox D, Shiboski CH, Schiødt M, Wu A, Lanfranchi H, Umehara H, Zhao Y, Challacombe S, Lam MY, de Souza Y, Schiødt J, Holm H, Bisio PAM, Gandolfo MS, Sawaki T, Li M, Zhang W, Varghese-Jacob B, Ibsen P, Keszler A, Kurose N, Nojima T, Odell E, Criswell LA, Jordan R, Greenspan JS, Sjögren’s International Collaborative Clinical Alliance Research Groups (2011) Associations between salivary gland histopathologic diagnoses and phenotypic features of Sjögren’s syndrome among 1,726 registry participants. Arthritis Rheum 63:2021–2030
American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias (2002) This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med 165:277–304
Andersen PK, Borgan O, Gill RD et al (1993) Statistical models based on counting processes, vol 18. Springer-Verlag, New York, p 322
Valesini G, Priori R, Bavoillot D, Osborn J, Danieli MG, Del Papa N et al (1997) Differential risk of non-Hodgkin’s lymphoma in Italian patients with primary Sjögren’s syndrome. J Rheumatol 24:2376–2380
Maciel G, Crowson CS, Matteson EL, Cornec D (2017) Incidence and mortality of physician-diagnosed primary Sjögren syndrome: time trends over a 40-year period in a population-based US cohort. Mayo Clin Proc 92(5):734–743. https://doi.org/10.1016/j.mayocp.2017.01.020
Singh AG, Singh S, Matteson EL (2016) Rate, risk factors and causes of mortality in patients with Sjögren’s syndrome: a systematic review and meta-analysis of cohort studies. Rheumatology (Oxford) 55(3):450–460. https://doi.org/10.1093/rheumatology/kev354
Yazısız V, Avcı AB, Erbasan F, Kiriş E, Terzioğlu E (2009) Diagnostic performance of minor salivary gland biopsy, serological and clinical data in Sjogren’s syndrome: a retrospective analysis. Rheumatol Int 29:403–409. https://doi.org/10.1007/s00296-008-0698-1
Yazısız V, Arslan G, Özbudak AH, Türker S, Erbasan F, Avcı AB, Ozbudak O, Terzioglu (2010) Lung involvement in patients with primary Sjögren’s syndrome: what are the predictors? Rheumatol Int 30:1317–1324. https://doi.org/10.1007/s00296-009-1152-8
Ramos-Casals M, Solans R, Rosas J, Camps MT, Gil A, Del Pino-Montes J, Alvo-Alen J, Jiménez-Alonso J, Micó ML, Beltrán J, Belenguer R, Pallarés L, GEMESS Study Group (2008) Primary Sjögren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine (Baltimore) 87(4):210–219. https://doi.org/10.1097/MD.0b013e318181e6af
Ibn Yacoub Y, Rostom S, Laatiris A, Hajjaj-Hassouni N (2012) Primary Sjögren’s syndrome in Moroccan patients: characteristics, fatigue and quality of life. Rheumatol Int 32(9):2637–2643. https://doi.org/10.1007/s00296-011-2009-5
Nannini C, Jebakumar AJ, Crowson CS, Ryu JH, Matteson EL (2013) Primary Sjogren’s syndrome 1976-2005 and associated interstitial lung disease: a population-based study of incidence and mortality. BMJ Open 3(11):e003569. https://doi.org/10.1136/bmjopen-2013-003569
Ito I, Nagai S, Kitaichi M, Nicholson AG, Johkoh T, Noma S, Kim DS, Handa T, Izumi T, Mishima M (2005) Pulmonary manifestations of primary Sjogren’s syndrome: a clinical, radiologic, and pathologic study. Am J Respir Crit Care Med 171:632–638
Enomoto Y, Takemura T, Hagiwara E, Iwasawa T, Fukuda Y, Yanagawa N, Sakai F, Baba T, Nagaoka S, Ogura T (2013) Prognostic factors in interstitial lung disease associated with primary Sjogren’s syndrome: a retrospective analysis of 33 pathologically-proven cases. PLoS One 8:e73774. https://doi.org/10.1371/journal.pone.0073774
Gao H, Zhang XW, He J, Zhang J, An Y, Sun Y, Jia RL, Li SG, Zhang LJ, Li ZG (2018) Prevalence, risk factors, and prognosis of interstitial lung disease in a large cohort of Chinese primary Sjögren syndrome patients: a case-control study. Medicine (Baltimore) 97(24):e11003. https://doi.org/10.1097/MD.0000000000011003
Roca F, Dominique S, Schmidt J, Smail A, Duhaut P, Lévesque H, Marie I (2017) Interstitial lung disease in primary Sjogren’s syndrome. Autoimmun Rev 16:48–54. https://doi.org/10.1016/j.autrev.2016.09.017
Chen MH, Chou HP, Lai CC, Chen YD, Chen MH, Lin HY, Huang DF (2014) Lung involvement in primary Sjogren’s syndrome: correlation between high-resolution computed tomography score and mortality. J Chin Med Assoc 77:75–82. https://doi.org/10.1016/j.jcma.2013.11.001
Rubio-Rivas M, Royo C, Simeón CP, Corbella X, Fonollosa V (2014) Mortality and survival in systemic sclerosis: systematic review and meta-analysis. Semin Arthritis Rheum 44(2):208–219. https://doi.org/10.1016/j.semarthrit.2014.05.010
Hyldgaard C, Hilberg O, Pedersen AB, Ulrichsen SP, Løkke A, Bendstrup E, Ellingsen T (2017) A population-based cohort study of rheumatoid arthritis-associated interstitial lung disease: comorbidity and mortality. Ann Rheum Dis 76(10):1700–1706. https://doi.org/10.1136/annrheumdis-2017-211138
Lazarus MN, Robinson D, Mak V, Moller H, Isenberg DA (2006) Incidence of cancer in a cohort of patients with primary Sjogren’s syndrome. Rheumatology (Oxford) 45:1012–1015
Theander E, Henriksson G, Ljungberg O, Mandl T, Manthorpe R, Jacobsson LTH (2006) Lymphoma and other malignancies in primary Sjogren’s syndrome: a cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis 65:796–803
Kassan SS, Thomas TL, Moutsopoulos HM, Hoover R, Kimberly RP, Budman DR, Costa J, Decker JL, Chused TM (1978) Increased risk of lymphoma in sicca syndrome. Ann Intern Med 89(6):888–892
Liang Y, Yang Z, Qin B, Zhong R (2014) Primary Sjogren’s syndrome and malignancy risk: a systematic review and meta-analysis. Ann Rheum Dis 73(6):1151–1156. https://doi.org/10.1136/annrheumdis-2013-203305
Flores-Chávez A, Kostov B, Solans R, Fraile G, Maure B, Feijoo-Massó C, Rascón FJ, Pérez-Alvarez R, Zamora-Pasadas M, García-Pérez A, Lopez-Dupla M, Duarte-Millán MÁ, Ripoll M, Fonseca-Aizpuru E, Guisado-Vasco P, Pinilla B, dela Red G, Chamorro AJ, Morcillo C, Fanlo P, Soto-Cárdenas MJ, Retamozo S, Ramos-Casals M, Brito-Zerón P, GEAS-SS SEMI Registry (2018) Severe, life-threatening phenotype of primary Sjögren’s syndrome: clinical characterisation and outcomes in 1580 patients (GEAS-SS Registry). Clin Exp Rheumatol 36 Suppl 112(3):121–129
Teh CL, Wan SA, Ling GR (2018) Severe infections in systemic lupus erythematosus: disease pattern and predictors of infection-related mortality. Clin Rheumatol 37(8):2081–2086. https://doi.org/10.1007/s10067-018-4102-6
This study is supported by Akdeniz University Scientific Research Projects Unit.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the World Medical Association Helsinki Declaration 2018 and its later amendments or comparable ethical standards. The study was approved by the Ethics Committee of Akdeniz University School of Medicine (Approval number:2019-534).
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
About this article
Cite this article
Yazisiz, V., Göçer, M., Erbasan, F. et al. Survival analysis of patients with Sjögren’s syndrome in Turkey: a tertiary hospital-based study. Clin Rheumatol 39, 233–241 (2020) doi:10.1007/s10067-019-04744-6
- Sjögren’s syndrome
- Standard mortality rates