Determining the association between systematic lupus erythematosus and the occurrence of primary biliary cirrhosis: a systematic review and meta-analysis.

Background: Autoimmune diseases often coexist; however, the concomitant occurrence of systemic lupus erythematosus (SLE) and primary biliary cirrhosis (PBC) is rare. Therefore, this study aims to provide a comprehensive summary of evidence regarding the co-occurrence of SLE and PBC.

Methods: PubMed, Web of Science, ScienceDirect , and Google Scholar databases were systematically and comprehensively searched for records published up to February 2024. Full-text articles that aligned with the study's aim were included, while those published in languages other than English and those designed as case reports, reviews, conference abstracts, or editorials were excluded. Statistical analyses were performed using Comprehensive Meta-Analysis software, and methodological quality was assessed using the Newcastle-Ottawa Scale.

Results: Only 14 studies that met the inclusion criteria with 3944 PBC and 9414 SLE patients were included for review and analysis. Pooled data analysis revealed that approximately 1.1% of SLE patients have concomitant PBC (range: 0.02-7.5%), while around 2.7% of PBC patients concurrently have SLE (range: 1.3-7.5%). Furthermore, qualitative data analysis indicated that the prevalence of PBC in SLE patients presenting with hepatic dysfunction or abnormal liver enzymes ranges from 2 to 7.5%.

Conclusion: Although the concomitant occurrence of SLE and PBC is rare, the small proportion of patients where these diseases coexist warrants close monitoring by clinicians. This underscores the importance of surveillance to prevent their co-occurrence.