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Survival analysis of patients with Sjögren’s syndrome in Turkey: a tertiary hospital-based study

Clinical Rheumatology volume 39, pages 233–241 (2020)Cite this article

Abstract

Objectives

This study was conducted to determine long-term survival rates and the factors associated with mortality in Turkish primary Sjögren syndrome (pSS) patients.

Methods

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.

Results

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.

Conclusions

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.

Key Point
• Mortality in Sjögren’s syndrome.

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Fig. 1

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Acknowledgments

This study is supported by Akdeniz University Scientific Research Projects Unit.

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Affiliations

  1. Department of Internal Medicine, Division of Rheumatology, Akdeniz Üniversitesi Hastanesi, Konyaaltı, Antalya, Turkey

    Veli Yazisiz, Funda Erbasan, İsmail Uçar, Bengisu Aslan, Şuayp Oygen, Edip Gökalp Gök & Mustafa Ender Terzioğlu

  2. Department of Internal Medicine, Division of Hematology, Antalya Education and Research Hospital, Antalya, Turkey

    Mesut Göçer

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  1. Veli Yazisiz
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  2. Mesut Göçer
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  3. Funda Erbasan
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  5. Bengisu Aslan
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Contributions

VY contributed to conception and design of the study, analysis and interpretation of data, and drafting the manuscript; MG, İU, BA, ŞO, EG and FE collected data and reviewed the literature; ET was responsible for the revision of the manuscript for important intellectual content; all authors issued approval for the final version to be submitted.

Corresponding author

Correspondence to Veli Yazisiz.

Ethics declarations

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).

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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). https://doi.org/10.1007/s10067-019-04744-6

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  • DOI: https://doi.org/10.1007/s10067-019-04744-6

Keywords

  • Mortality
  • Prognosis
  • Sjögren’s syndrome
  • Standard mortality rates
  • Survival