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Association of immunological features with clinical manifestations in primary Sjogren’s syndrome: a single-center cross-sectional study

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Abstract

The objectives of this study were to describe the clinical features and evaluate the utility of immunological features as predictors of organ involvement and disease severity in patients with primary Sjogren’s syndrome (pSS). In this single-center observational cross-sectional study, subjects fulfilling the 2012 AECG criteria or 2016 ACR/EULAR criteria for pSS were included. Details of glandular, extra-glandular manifestations, ESSDAI, ESSPRI, ANA, anti-Ro/La antibodies, rheumatoid factor (RF), complement (C3 and C4) levels and hyperglobulinemia were noted. Chi-square and Mann–Whitney U tests were performed for determining associations and relative risk (RR) was calculated. Sixty-four subjects with pSS were included in the study. Females constituted 92% and median age at onset was 37.5 (15–74) years. Ocular or oral sicca was noted in 61 (95.3%) subjects and parotidomegaly was noted in 17 (26.5%) subjects. Extra-glandular manifestations noted were: constitutional (85.9%), articular (65.6%), renal (29.6%), hematological (26.6%), cutaneous (12.5%), peripheral nerves (9.3%) and pulmonary (4.7%). Immunological features noted were: ANA (85.9%), anti-Ro (81.3%), anti-La (60.9%), RF (84.4%), hypocomplementemia (39.1%) and hyperglobulinemia (62.5%). Median ESSDAI was 6 (0–23) and ESSPRI was 7 (0–10). ANA was associated with younger age and renal involvement (RR 1.25). Anti-Ro was associated with younger age, renal involvement (RR 1.36) and high ESSDAI. Anti-La was associated with high renal (RR 3.4) and low articular involvement (RR 2.75). RF was associated with hematological involvement and hyperglobulinemia was associated with younger age. Certain immunological features can help predict the organ involvement in patients with pSS. Larger, prospective follow-up studies are needed to clearly understand these associations.

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The study data will be provided on contacting the corresponding author.

References

  1. Mahendranath KM. Sjogren’s syndrome-diagnosis and management. J Assoc Physicians India. 2006;54(Suppl):54–7.

    PubMed  Google Scholar 

  2. Mavragani CP, Moutsopoulos HM. The geoepidemiology of Sjogren’s syndrome. Autoimmun Rev. 2010;9:A305–10.

    Article  Google Scholar 

  3. Sandhya P, Jayaseelan L, Scofield RH, Danda D. Clinical characteristics and outcome of primary Sjogren’s syndrome: A large Asian Indian cohort. Open Rheumatol J. 2015;9:36–45.

    Article  CAS  Google Scholar 

  4. Locht H, Pelck R, Manthorpe R. Clinical manifestations correlated to the prevalence of autoantibodies in a large (n=321) cohort of patients with primary Sjögren’s syndrome: a comparison of patients initially diagnosed according to the Copenhagen classification criteria with the American-European consensus criteria. Autoimmun Rev. 2005;4:276–81.

    Article  CAS  Google Scholar 

  5. Martel C, Gondran G, Launay D, et al. Active immunological profile is associated with systemic Sjögren’s syndrome. J Clin Immunol. 2011;31:840–7.

    Article  CAS  Google Scholar 

  6. Pertovaara M, Pukkala E, Laippala P, Miettinen A, Pasternack A. A longitudinal cohort study of Finnish patients with primary Sjögren’s syndrome: clinical, immunological, and epidemiological aspects. Ann Rheum Dis. 2001;60:467–72.

    Article  CAS  Google Scholar 

  7. Ramos-Casals M, Solans R, Rosas J, et al. Primary Sjögren’s syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine (Baltimore). 2008;87:210–9.

    Article  CAS  Google Scholar 

  8. Lin DF, Yan SM, Zhao Y, et al. Clinical and prognostic characteristics of 573 cases of primary Sjögren’s syndrome. Chin Med J (Engl). 2010;123:3252–7.

    Google Scholar 

  9. Alexander EL, Arnett FC, Provost TT, Stevens MB. Sjögren’s syndrome: association of anti-Ro (SS-A) antibodies with vasculitis, hematologic abnormalities, and serologic hyperreactivity. Ann Intern Med. 1983;98:155–9.

    Article  CAS  Google Scholar 

  10. Cho CS, Park SH, Min JK, Lee SH, Kim HY. Clinical significances of antibodies to Ro/SS-A autoantigens and its subtypes in primary Sjogren’s syndrome. Korean J Intern Med. 1997;12:176–81.

    Article  CAS  Google Scholar 

  11. Ramos-Casals M, Brito-Zeron P, Yague J, et al. Hypocomplementaemia as an immunological marker of morbidity and mortality in patients with primary Sjogren’s syndrome. Rheumatology (Oxford). 2005;44:89–94.

    Article  CAS  Google Scholar 

  12. Hernández-Molina G, Leal-Alegre G, Michel-Peregrina M. The meaning of anti-Ro and anti-La antibodies in primary Sjögren’s syndrome. Autoimmun Rev. 2011;10:123–5.

    Article  CAS  Google Scholar 

  13. Baldini C, Pepe P, Quartuccio L, et al. Primary Sjögren’s syndrome as a multi-organ disease: impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients. Rheumatology (Oxford). 2014;53:839–44.

    Article  Google Scholar 

  14. Brito-Zerón P, Acar-Denizli N, Ng WF, et al. How immunological profile drives clinical phenotype of primary Sjögren’s syndrome at diagnosis: analysis of 10,500 patients (Sjögren big data project). Clin Exp Rheumatol. 2018;36(Suppl. 112):102–12.

    PubMed  Google Scholar 

  15. Brito-Zerón P, Ramos-Casals M, Bove A, Sentis J, Font J. Predicting adverse outcomes in primary Sjögren’s syndrome: identification of prognostic factors. Rheumatology (Oxford). 2007;46:1359–62.

    Article  Google Scholar 

  16. Retamozo S, Akasbi M, Brito-Zerón P, et al. Anti-Ro52 antibody testing influences the classification and clinical characterisation of primary Sjögren’s syndrome. Clin Exp Rheumatol. 2012;30:686–92.

    PubMed  Google Scholar 

  17. Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjogren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus group. Ann Rheum Dis. 2002;61:554–8.

    Article  CAS  Google Scholar 

  18. Shiboski CH, Shiboski SC, Seror R, et al. 2016 ACR-EULAR classification criteria for primary Sjögren’s syndrome: a consensus and data-driven methodology involving three international patient cohorts. Arthritis Rheumatol. 2017;69:35–45.

    Article  Google Scholar 

  19. Seror R, Ravaud P, Bowman SJ, et al. EULAR Sjogren’s syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjogren’s syndrome. Ann Rheum Dis. 2010;69:1103–9.

    Article  Google Scholar 

  20. Mohanasundaram K, Mani M, Chinnadurai S, et al. Study on demography and outcome of extraglandular manifestations of primary Sjögren’s syndrome. Indian J Rheumatol. 2016;11:202–6.

    Article  Google Scholar 

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Acknowledgements

We thank Mr. Prabir Dass, Mrs. Rajwant Kaur and Mrs. Shashi Anand, Department of Immunopathology, for their help in performing various laboratory investigations of study subjects.

Funding

No funding was received from any source to carry out the work described in this study.

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Authors and Affiliations

  1. Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India

    Hitesh Srinivasa Krishna Relangi, G. S. R. S. N. K. Naidu, Varun Dhir, Shefali K. Sharma, Aman Sharma & Sanjay Jain

  2. Department of Internal Medicine, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India

    Vikas Sharma

  3. Department of Immunopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

    Mahendra Kumar & Ranjana W. Minz

Authors
  1. Hitesh Srinivasa Krishna Relangi
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  2. G. S. R. S. N. K. Naidu
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  3. Vikas Sharma
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  4. Mahendra Kumar
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  5. Varun Dhir
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  6. Shefali K. Sharma
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  7. Aman Sharma
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  8. Ranjana W. Minz
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  9. Sanjay Jain
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Contributions

HSKR and GN conceptualized the study; HSKR, GN, VS, VD, SKS, AS and SJ contributed in collecting clinical data; MK and RWM contributed in collecting immunological data. HSKR and GN analyzed and interpreted the data. HSKR and GN drafted the manuscript while all other authors critically analyzed and revised the manuscript. All authors approved the final version of the manuscript for submission and agree to be accountable for all aspects of the work being submitted.

Corresponding author

Correspondence to G. S. R. S. N. K. Naidu.

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The authors have no relevant financial or non-financial interests to disclose.

Ethics approval

The study was conducted after approval by the Institute Ethics Committee and the study complies with the Declaration of Helsinki. A written informed consent was obtained from all subjects before being enrolled in the study.

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Relangi, H.S.K., Naidu, G.S.R.S.N.K., Sharma, V. et al. Association of immunological features with clinical manifestations in primary Sjogren’s syndrome: a single-center cross-sectional study. Clin Exp Med 22, 613–620 (2022). https://doi.org/10.1007/s10238-021-00772-2

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  • DOI: https://doi.org/10.1007/s10238-021-00772-2

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