Cardiac involvement in patients with primary biliary cholangitis: A 14-year longitudinal survey-based study

Patients with primary biliary cholangitis (PBC) can have extrahepatic manifestations. However, data about cardiac involvement of PBC is limited. We aimed in this study to analyze the clinical characteristics in patients with PBC complicated with and without cardiac involvement, and the risk factors of cardiac involvement in PBC. PBC patients admitted to Peking Union Medical College Hospital between January 2002 and February 2016 were consecutively enrolled. Structured interview, systemic rheumatologic examination, and laboratory tests were conducted for each patient, and risk factors of cardiac involvement were analyzed by comparing patients with and without cardiac involvement. In total, 580 PBC patients were enrolled, and cardiac involvement was identified in 24 patients (4%), with 11 male (46%) and a mean age of 57±8 year. Cardiomyopathy and arrhythmias were presented in 17 (70.8%) and 21 (87.5%) patients, respectively. Patients with cardiac involvement were more frequently male (46% vs. 11%, P<0.01), complicated with inflammatory myopathy (IM) (58% vs. 1%, P<0.01), and had a longer disease course (median, 72 vs 24 month, P<0.01). Furthermore, concomitant IM was the independent risk factor of cardiac involvement in PBC (OR = 77.333, 95% CI: 23.704-252.294). Cardiac involvement was a rare complication of PBC, which was more frequently observed in male or long-course patients. Importantly, concomitant IM was the strong independent risk factor of cardiac involvement in PBC. Given cardiac involvement is a serious complication, thorough evaluation of cardiac manifestation in high-risk PBC patients is highly recommended.