Razavi Journal of Medicine

Razavi Journal of Medicine

Association between primary biliary cholangitis and risk of Sjögren's syndrome: a systematic review and meta-analysis

Document Type : Review Article/ Systematic Review Article/ Meta Analysis

Authors
Division of Rheumatology, Department of Internal Medicine, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.
Abstract
Background: Primary biliary cholangitis (PBC) is associated with certain autoimmune diseases.

Objectives: This study aims to investigate the association between PBC and the risk of Sjögren's syndrome by applying a systematic review and meta-analysis.

Method: Databases such as ProQuest, PubMed, Web of Science, Cochrane, and Google Scholar were searched up to July 17, 2024, using keywords like "Sjögren's Syndrome," "Sicca Syndrome," "Liver Cirrhosis, Biliary," "Primary Biliary Cirrhosis," "Primary Biliary Cholangitis," "Obstructive Liver Cirrhosis," and their MeSH equivalents. No time or language restrictions were applied to the search. Studies directly addressing the association between PBC and the risk of Sjögren's syndrome were included. For low heterogeneity, a fixed-effects model was used. For moderate and severe heterogeneity, a random-effects model was used. Also, I²=85.6% for cohort studies, I²=0% for Mendelian randomization studies, and I²=0% for case-control studies. STATA 14 software was used for data analysis, and P<0.05 was considered significant.

Results: In the 8 studies reviewed (two case-control studies, four Mendelian randomization studies, and two cohort studies), primary biliary cholangitis increased the risk of Sjögren's syndrome (OR: 1.17, (95%CI: 1.11, 1.25)), systemic lupus erythematosus (OR: 1.42, (95%CI: 1.35, 1.49)), and rheumatoid arthritis (OR: 1.14, (95%CI: 1.03, 1.27)). However, there was no statistically significant correlation between PBC and the risk of psoriasis (OR: 1.45, 95% CI: 0.71, 2.95) or systemic sclerosis (OR: 1.23, 95% CI: 0.96, 1.58). Sjögren's syndrome increased the risk of PBC (OR: 1.73, 95% CI: 1.12, 2.70). In Mendelian randomization studies (OR: 1.17, 95% CI: 1.13, 1.21) and case-control studies (OR: 8.47, 95% CI: 1, 71.58), PBC was shown to increase the risk of Sjögren's syndrome; however, cohort studies did not find a significant association between PBC and the risk of Sjögren's syndrome (OR: 1.43, 95% CI: 0.19, 10.69).

Conclusions: PBC is recognized as a risk factor for rheumatoid arthritis, Sjögren's syndrome, and systemic lupus erythematosus; moreover, there is a significant risk that Sjögren's syndrome will lead to PBC.
Keywords

Acknowledgments: We wish to thank all our colleagues at Isfahan University of Medical Sciences.


Conflict of interest: The authors declare that they have no conflict of interest. 


Availability of data and materials: This is a review study, and it is not an original. Data availability is the corresponding author's responsibility.


Ethical approval: This article does not contain any studies with human participants or animals performed by any of the authors.


Consent for publication: Not applicable.


Funding: None


Author contributions: All authors contributed to the study conception and design, data collection, data analysis and interpretation, and manuscript preparation. All authors reviewed and approved the final version of the manuscript.

Open Access Policy: This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. To view a copy of this licence, visit https://creativecommons.org/licenses/by/4.0/

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Available Online from 10 August 2026