Endothelin-1 and Anticardiolipin Antibodies in Primary Biliary Cholangitis Patients with Portal Hypertension: A Pilot Retrospective Study

Primary biliary cholangitis (PBC) is a chronic autoimmune, cholestatic liver disease leading to fibrosis, cirrhosis, and portal hypertension. Endothelin-1 (ET-1), a potent vasoconstrictor with profibrotic properties, has been implicated in the pathogenesis of liver fibrosis and portal hypertension. Anti-cardiolipin antibodies (aCL) may further contribute to disease progression by promoting thrombotic complications. Our aim was to evaluate serum ET-1 concentrations and anti-cardiolipin antibody levels in PBC patients and their association with portal hypertension. Serum samples from 80 patients with PBC, including patients with portal hypertension, and 25 healthy controls were analyzed. ET-1 concentrations and anti-cardiolipin antibodies were measured using commercially available immunoassays. Elevated serum ET-1 levels were detected in 84% of patients with PBC and portal hypertension and the ET-1 concentrations were also significantly higher in this group compared to the control group. Serum ET-1 levels positively correlated with total bilirubin concentrations and the severity of histological fibrosis. Anti-cardiolipin antibodies were detected in 32% of PBC patients with portal hypertension. Elevated serum ET-1 concentrations and the presence of anti-cardiolipin antibodies are associated with advanced liver disease and portal hypertension in patients with PBC. These biomarkers may have potential value in identifying patients at increased risk of disease progression and worsening liver function.