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The risk of developing non-Hodgkin lymphoma and the mortality rate in primary Sjögren’s syndrome: a single-centre, retrospective cohort study

  • Cohort Studies
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Abstract

Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disease that is manifested by the sensation of dry eyes and dry mouth. The higher incidence of non-Hodgkin lymphoma (NHL) among pSS has already been extensively researched. However, there are uncertanties whether the mortality risk in pSS patients and in pSS patients with NHL is increased. The purpose of this study was to describe the prevalence of NHL among pSS patients and to calculate their mortality risk. We retrospectively analysed data on 1367 patients treated in our rheumatology department under the ICD-10 code M35.0. The study finally recruited 155 patients who met the 2016 ACR/EULAR criteria for the diagnosis of pSS. Descriptive statistics was used in data analysis. We applied the indirect standardization by age to compare the incidence rate of NHL in our cohort to general population. Additionally, we compared the mortality in our study to the general population by calculating the standardized mortality ratio (SMR). The overall incidence rate of NHL was 440 per 100,000 patient-years. The SIR compared to the general population was 30.13 (95% CI 12.87–54.63). The overall mortality rate of pSS patients in our cohort was nearly identical to that of the general population (SMR = 0.98 [95% CI (0.47–1.69)]). This study confirms that there are significant differences in lymphoma prevalence, histology, and prognosis across the studied populations. Furthermore, this study found that patients with pSS have similar mortality risk as the general population, and no patient in our cohort died from NHL.

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Data availability

The data that support the findings of this study are not openly available due to reasons of sensitivity and are available from the corresponding author upon reasonable request. Data are located in controlled access data storage at Division of Clinical Immunology and Rheumatology, Department of Internal Medicine, University Hospital Centre Zagreb.

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Acknowledgements

We greatly appreciate the help from prof. Anamarija Jazbec in providing statistical assistance and guidance.

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Authors and Affiliations

  1. Division of Clinical Immunology and Rheumatology, Department of Internal Medicine, University Hospital Center Zagreb, 10000, Zagreb, Croatia

    Miroslav Mayer, Matea Martinic & Branimir Anic

  2. School of Medicine, University of Zagreb, 10000, Zagreb, Croatia

    Miroslav Mayer & Branimir Anic

  3. Department of Physichal Medicine and Rheumatology, National Memorial Hospital Vukovar, 32000, Vukovar, Croatia

    Mirna Reihl-Crnogaj

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  1. Miroslav Mayer
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Contributions

MM and BA contributed to the study conception and design. Material preparation, data collection and analysis were performed by MM and MRC. The first draft of the manuscript was written by MM and MM, and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript. All authors agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

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Correspondence to Matea Martinic.

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Conflict of interest

All authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Ethical approval

The study was approved by the Ethics Committee of the University Hospital Centre Zagreb, in Zagreb, Croatia (date: February 24, 2020; Protocol Number–Class: 8.1-20/32-2, Number: 02/21 AG).

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Mayer, M., Martinic, M., Reihl-Crnogaj, M. et al. The risk of developing non-Hodgkin lymphoma and the mortality rate in primary Sjögren’s syndrome: a single-centre, retrospective cohort study. Rheumatol Int 43, 2031–2036 (2023). https://doi.org/10.1007/s00296-023-05386-3

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