Autoimmune Hepatic Involvement in Systemic Sclerosis- Systematic Review and Meta Analysis

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Abstract

Introduction- Systemic sclerosis (SSc) is a heterogeneous, multisystem autoimmune disease characterized by fibrosis in genetically predisposed individuals. It can involve multiple organ systems, but hepatic involvement is rare. Methods- PubMed, Cochrane, Google Scholar, and Embase were searched for liver disease in SSc, and 11 studies were selected for analysis. Cochrane risk of bias tools and meta-analysis online were used. Controls were taken from the prevalence in the respective countries around the same time.Results- Among 11 studies including 2,797 patients with systemic sclerosis (SSc), 190 (6.7%) had liver disease, with 157 (5.6%) attributed to autoimmune causes. Primary biliary cholangitis (PBC) was the most common (5%), followed by autoimmune hepatitis (AIH) at 1.2%. PSC was only seen in 1 patient across the studies. There was a significant association in prevalence compared to the general population. Antimitochondrial antibodies (AMA) were positive in 10.87%, and anti-sp100 in 3.1%. Discussion-PBC was observed in approximately 5.6% of SSc patients—significantly higher than the general population prevalence of 0.02% to 0.04%—highlighting a strong association. This link is further supported by the presence of antimitochondrial antibodies (AMA) in 10.87% of patients, suggesting a high rate of subclinical or evolving PBC in this population. Autoimmune hepatitis (AIH) was identified in 1.2% of SSc patients. This rate exceeds general population estimates (0.02%–0.05%), suggesting that SSc may predispose patients to broader autoimmune liver involvement beyond PBC. In addition to AMA, anti-sp100 (3.1%) and anti-gp210 antibodies were frequently observed.

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