Background:Primary biliary cirrhosis (PBC) is a rare slowly progressive autoimmune disease characterized by inflammatory destruction and fibrosis of intrahepatic bile ducts. It is known to coexist together with rheumatological conditions such as Sjögren’s syndrome (SS) and systemic sclerosis (SSc). There is a wide range in reported prevalence of disease overlap with these entities; however, the exact prevalence rates remain unclear.Objectives:The objectives were to determine the prevalence of: 1) PBC in patients with SS and SSc (and the subsets of limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc)), 2) SSc and SS in patients with PBC, and 3) to analyze changes in frequency over time. SSc occurs in 3/10,000 and PBC in 4-40/10,000 but these rare autoimmune diseases are known to coexist together. We speculated that there could be more cases diagnosed due to increasing availability of standardized antibody tests such as ANA, centromere antibodies, ENA and mitochondrial antibodies.Methods:A systematic review of the literature was performed using Medline, EMBASE, CINAHL, and the Cochrane Library databases up till June 16, 2020. Only full text articles in the English language with at least 40 patients were included. Cohorts, case series, cross-sectional studies, correspondences and registries with reported prevalence rates of both PBC in patients with SS and SSc as well as SSc and SS in patients with PBC were included. Data on frequency of co-existent diseases was studied by year of publication to determine if prevalence changed over time using linear regression. We used the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) checklist to assess the quality of the studies.Results:Of 2876 citations identified, 67 were included in the analysis (n=33 for PBC, 15 for SSc, 18 for SS and 1 for SSc/SS). STROBE checklist scores ranged from 7-21. The prevalence of PBC was 5% in patients with SSc. Within the subsets, the prevalence of PBC in lcSSc was 8% and in dcSSc was 1%. In patients with SS, the prevalence of PBC was 4%. The prevalence of SSc overall in those with PBC was 5% and, within the subsets was 6% in lcSSc and 0% in dcSSc. The prevalence of SS in PBC was 18%. There was also no significant association between year of publication and prevalence. There was a lack of standardized definitions so misclassification may have occurred.Conclusion:PBC is increased in SSc but mostly in the lcSSc subset. SS in PBC is common at nearly 1 in 5. Over the years, there was no change in the prevalence of PBC in SSc indicating stability over time.Acknowledgements:Meagan Stanley, Western University Librarian.Disclosure of Interests:None declared.
A rare case of systemic sclerosis complicated with multiple autoimmune diseases (Sjögren's syndrome, Graves' disease, and primary biliary cirrhosis)
