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Sjögren’s disease activity associates with cardiovascular disease and monoclonal gammopathy: a university cohort study of disease activity and comorbidities

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Abstract

Background

We used the University of Wisconsin cohort to determine the extent to which the EULAR Sjögren’s syndrome disease activity index (ESSDAI) was associated with comorbidities that contribute to mortality.

Methods

Our University of Wisconsin, Madison cohort had 111 patients with Sjögren’s Disease (SjD) by 2016 ACR/EULAR criteria and 194 control patients with sicca. Our study was performed from March 1st, 2020 through April 1st, 2023. We collected data using a standardized collection tool, including components of the Charlson Comorbidity Index (CCI). Stratifying our SjD patients by ESSDAI < 5 and ESSDAI ≥ 5, we assessed differences in comorbidities associated with mortality.

Results

At time of SjD diagnosis, the ESSDAI ≥ 5 group had increased odds of peripheral vascular disease compared to controls (OR 10.17; 95% CI 1.18–87.87). Patients with a current ESSDAI ≥ 5 were more likely to have a myocardial infarction compared to controls (OR 9.87; 95% CI 1.17–83.49). SjD patients had increased prevalence of monoclonal gammopathy compared to controls (9.3% vs 0.5%, p < 0.001). SjD patients with high ESSDAI at diagnosis had greater prevalence of monoclonal gammopathy compared to the SjD patients with a low ESSDAI (16% vs 5%, p = .04). As reported elsewhere, the ESSDAI ≥ 5 group had increased odds of chronic pulmonary disease (OR 4.37; 95% CI 1.59–11.97).

Conclusion

We found high ESSDAI scores were associated with worse cardiovascular outcomes, specifically peripheral vascular disease and myocardial infarction. Furthermore, monoclonal gammopathy was more frequent in SjD patients compared to sicca controls, supporting screening for monoclonal gammopathy in the appropriate clinical scenario.

Key Points

High ESSDAI scores are associated with worse cardiovascular outcomes, specifically peripheral vascular disease and myocardial infarction.

Monoclonal gammopathy is more frequent in SjD patients than sicca controls, supporting screening for monoclonal gammopathy in the appropriate clinical scenario.

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Data availability

The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.

References

  1. Negrini S, Emmi G, Greco M, Borro M, Sardanelli F, Murdaca G et al (2022) Sjögren’s syndrome: a systemic autoimmune disease. Clin Exp Med 22(1):9–25. https://doi.org/10.1007/s10238-021-00728-6

    Article  PubMed  CAS  Google Scholar 

  2. Charlson ME, Pompei P, Ales KL, MacKenzie CR (1987) A new method of classifying prognostic comorbidity in longitudinal studies: development and validation. J Chronic Dis 40(5):373–383. https://doi.org/10.1016/0021-9681(87)90171-8

    Article  PubMed  CAS  Google Scholar 

  3. Brusselaers N, Lagergren J (2017) The Charlson Comorbidity Index in Registry-based Research. Methods Inf Med 56(5):401–406. https://doi.org/10.3414/me17-01-0051

    Article  PubMed  Google Scholar 

  4. Sundararajan V, Henderson T, Perry C, Muggivan A, Quan H, Ghali WA (2004) New ICD-10 version of the Charlson comorbidity index predicted in-hospital mortality. J Clin Epidemiol 57(12):1288–1294. https://doi.org/10.1016/j.jclinepi.2004.03.012

    Article  PubMed  Google Scholar 

  5. Seror R, Theander E, Brun JG, Ramos-Casals M, Valim V, Dorner T et al (2015) Validation of EULAR primary Sjogren’s syndrome disease activity (ESSDAI) and patient indexes (ESSPRI). Ann Rheum Dis 74(5):859–866. https://doi.org/10.1136/annrheumdis-2013-204615

    Article  PubMed  Google Scholar 

  6. Seror R, Bowman SJ, Brito-Zeron P, Theander E, Bootsma H, Tzioufas A et al (2015) EULAR Sjogren’s syndrome disease activity index (ESSDAI): a user guide. RMD Open 1(1):e000022. https://doi.org/10.1136/rmdopen-2014-000022

    Article  PubMed  PubMed Central  Google Scholar 

  7. Seror R, Ravaud P, Mariette X, Bootsma H, Theander E, Hansen A et al (2011) EULAR Sjogren’s Syndrome Patient Reported Index (ESSPRI): development of a consensus patient index for primary Sjogren’s syndrome. Ann Rheum Dis 70(6):968–972. https://doi.org/10.1136/ard.2010.143743

    Article  PubMed  Google Scholar 

  8. 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

    Article  PubMed  Google Scholar 

  9. Brito-Zerón P, Kostov B, Solans R, Fraile G, Suárez-Cuervo C, Casanovas A et al (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

    Article  PubMed  CAS  Google Scholar 

  10. Brito-Zerón P, Flores-Chávez A, Horváth IF, Rasmussen A, Li X, Olsson P et al (2023) Mortality risk factors in primary Sjögren syndrome: a real-world, retrospective, cohort study. EClinicalMedicine 61:102062. https://doi.org/10.1016/j.eclinm.2023.102062

    Article  PubMed  PubMed Central  Google Scholar 

  11. Papageorgiou A, Ziogas DC, Mavragani CP, Zintzaras E, Tzioufas AG, Moutsopoulos HM et al (2015) Predicting the outcome of Sjogren's syndrome-associated non-Hodgkin's lymphoma patients. PLoS One 10(2). https://doi.org/10.1371/journal.pone.0116189

  12. Zippel CL, Beider S, Kramer E, Konen FF, Seeliger T, Skripuletz T et al (2022) Premature stroke and cardiovascular risk in primary Sjögren’s syndrome. Front Cardiovasc Med 9:1048684. https://doi.org/10.3389/fcvm.2022.1048684

    Article  PubMed  PubMed Central  Google Scholar 

  13. Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM et al (2017) 2016 American college of rheumatology/european league against rheumatism classification criteria for primary sjogren’s syndrome: A consensus and data-driven methodology involving three international patient cohorts. Arthritis Rheumatol 69(1):35–45. https://doi.org/10.1002/art.39859

    Article  PubMed  Google Scholar 

  14. Seror R, Gottenberg JE, Bootsma H, Saraux A, Theander E, Ramos-Casals M et al (2014) Defining disease activity sates and minimal clinically important improvement (Mcii) with the eular primary sjogren’s syndrome disease activity index (Essdai). Ann Rheum Dis 73:144–145. https://doi.org/10.1136/annrheumdis-2014-eular.4399

    Article  Google Scholar 

  15. Cafaro G, Perricone C, Riccucci I, Bursi R, Calvacchi S, Alunno A et al (2021) Traditional and disease-related non-computed variables affect algorithms for cardiovascular risk estimation in Sjögren’s syndrome and rheumatoid arthritis. Clin Exp Rheumatol 39 Suppl 133(6):107–13. https://doi.org/10.55563/clinexprheumatol/xef8uz

    Article  PubMed  Google Scholar 

  16. Łuczak A, Małecki R, Kulus M, Madej M, Szahidewicz-Krupska E, Doroszko A (2021) Cardiovascular risk and endothelial dysfunction in primary sjogren syndrome is related to the disease activity. Nutrients 13(6). https://doi.org/10.3390/nu13062072

  17. Chabert P, Danjou W, Mezidi M, Berthiller J, Bestion A, Fred AA et al (2021) Short- and long-term prognosis of acute critically ill patients with systemic rheumatic diseases: A retrospective multicentre study. Medicine (Baltimore) 100(35):e26164. https://doi.org/10.1097/md.0000000000026164

    Article  PubMed  CAS  Google Scholar 

  18. Atisha-Fregoso Y, Rivera-Vicencio Y, Baños-Pelaez M, Hernández-Molina G (2015) Main causes and risk factors for hospitalisation in patients with primary Sjögren’s syndrome. Clin Exp Rheumatol 33(5):721–725

    PubMed  Google Scholar 

  19. Sandoval-Flores MG, Chan-Campos I, Hernández-Molina G (2021) Factors influencing the EULAR Sjögren’s Syndrome Patient-Reported Index in primary Sjögren’s syndrome. Clin Exp Rheumatol 39 Suppl 133(6):153–8. https://doi.org/10.55563/clinexprheumatol/mvcai5

    Article  PubMed  Google Scholar 

  20. Bai Z, Hu C, Zhong J, Dong L (2023) Prevalence and risk factors of monoclonal gammopathy in patients with autoimmune inflammatory rheumatic disease: A systematic review and meta-analysis. Mod Rheumatol 33(4):792–802. https://doi.org/10.1093/mr/roac066

    Article  PubMed  Google Scholar 

  21. Tomi AL, Belkhir R, Nocturne G, Desmoulins F, Berge E, Pavy S et al (2016) Brief report: Monoclonal gammopathy and risk of lymphoma and multiple myeloma in patients with primary sjögren’s syndrome. Arthritis Rheumatol 68(5):1245–1250. https://doi.org/10.1002/art.39534

    Article  PubMed  Google Scholar 

  22. Zhong H, Liu S, Wang Y, Xu D, Li M, Zhao Y et al (2022) Primary Sjögren’s syndrome is associated with increased risk of malignancies besides lymphoma: A systematic review and meta-analysis. Autoimmun Rev 21(5):103084. https://doi.org/10.1016/j.autrev.2022.103084

    Article  PubMed  Google Scholar 

  23. Heus A, Arends S, Van Nimwegen JF, Stel AJ, Nossent GD, Bootsma H (2020) Pulmonary involvement in primary Sjögren’s syndrome, as measured by the ESSDAI. Scand J Rheumatol 49(1):38–46. https://doi.org/10.1080/03009742.2019.1634221

    Article  PubMed  CAS  Google Scholar 

  24. Zeher M, Horvath IF, Szanto A, Szodoray P (2009) Autoimmune thyroid diseases in a large group of Hungarian patients with primary Sjögren’s syndrome. Thyroid 19(1):39–45. https://doi.org/10.1089/thy.2007.0398

    Article  PubMed  CAS  Google Scholar 

  25. Alfaris N, Curiel R, Tabbara S, Irwig MS (2010) Autoimmune thyroid disease and Sjögren syndrome. J Clin Rheumatol 16(3):146–147. https://doi.org/10.1097/RHU.0b013e3181d52a28

    Article  PubMed  Google Scholar 

  26. Kaplan MJ, Ike RW (2002) The liver is a common non-exocrine target in primary Sjögren’s syndrome: a retrospective review. BMC Gastroenterol 2:21. https://doi.org/10.1186/1471-230x-2-21

    Article  PubMed  PubMed Central  Google Scholar 

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Acknowledgements

This work was supported by the Clinical and Translational Science Award (CTSA) program, through the NIH National Center for Advancing Translational Sciences (NCATS), grant 1KL2TR002374. The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH

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Authors

Contributions

BB analyzed, interpreted data, and was a major contributor to writing the manuscript. HD helped with data extraction, interpreting data, and contributed to writing the manuscript. TB helped with data extraction. KH helped with defining and identifying cohort and contributed in review of manuscript, SM helped with defining and identifying cohort, data analysis and writing of manuscript. All authors read, approved the final manuscript, and take responsibility for the content of this manuscript.

Corresponding author

Correspondence to Sara S. McCoy.

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Financial disclosures

Support: This work was supported by the Clinical and Translational Science Award (CTSA) program, through the NIH National Center for Advancing Translational Sciences (NCATS), grant 1KL2TR002374. The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH.

Hunter S. Dowds received a summer research award from the Herman and Gwendolyn Shapiro Foundation in support of the research he provided.

Competing interests

All authors in this study deny relevant financial and nonfinancial competing interests. SSM receives unrelated consulting fees from Novartis, BMS, Otsuka, Visterra, Horizon, Kiniksa, Target RWE, iCell.

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Bohman, B.R., Dowds, H.S., Blagogee, T.E. et al. Sjögren’s disease activity associates with cardiovascular disease and monoclonal gammopathy: a university cohort study of disease activity and comorbidities. Clin Rheumatol 43, 1093–1101 (2024). https://doi.org/10.1007/s10067-024-06890-y

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