Abstract: Objective To evaluate the clinical characteristics and prognostic factors in patients with primary biliary cholangitis (PBC), focusing on those with concurrent autoimmune diseases (ATD) in the PBC patient population.Methods A retrospective analysis was conducted on 152 PBC patients,who were admitted between September 2008 and December 2018. The cohort consisted of 17 males and 135 females, aged 52 (41, 64) years. All patients met the diagnositic criterria for PBC and received treatment with ursodeoxycholic acid(UDCA). Clinical data and prognostic outcomes between PBC patients without concurrent autoimmune diseases and those with concurrent autoimmune hepatitis(PBC-AIH) or Sjogren Syndrome(PBC-SS) were compared.Results Out of 152 evaluated cases of PBC, 104 cases were identified as isolated PBC (68.4%), 11 as PBC-AIH(7.2%), and 24 as PBC-SS (15.8%). Additionally, 3 patients were diagnosed with rheumatoid arthritis (2.0%), 2 with systemic lupus erythematosus, 2 with Graves' disease and 2 with coexisting AIH and SS (1.3%). In comparing isolated PBC with PBC-AIH, the PT was significantly different at 12.0 (11.1, 13.9) s and 12.8 (11.7, 17.3) s, respectively(P<0.05) Similarly, ANA(+) was significantly different between isolated PBC and PBC-AIH, with 55 cases (52.9%) and 9 cases (81.8%), respectively (P<0.05). Furthermore, comparisons of WBC, Hb, Alb and HDL levels were made between isolated PBC and PBC-SS groups. In the isolated PBC group, these values were 4.5 (3.4, 6.2)×10⁹/L, 114.2 (106.1, 128.4) g/L, 34.6 (29.8, 38.7) g/L and 1.1 (1.0, 1.5) mmol/L. 17.0) g/L,respectively. In the PBS-SS group, these values were 3.2 (2.6, 4.2)×10⁹/L, 111.2 (79.8, 117.0) g/L, 33.3 (28.2, 36.5) g/L and 0.9 (0.5, 1.2) mmol/L] respectively, revealing statistically significant differences(P<0.05). Survival data were collected up to January 1st, 2022. The median survival times for patients with isolated PBC, PBC-AIH and PBC-SS were 72.0, 58.5 and 50.0 months, respectively. The median survival time of patients with isolated PBC was significantly longer than those with PBC-AIH and PBC-SS (P<0.05). Similarly, the 5-year survival rate for patients with isolated PBC (80.8%) was significantly higher than for patients with(63.6%) and PBC-SS(58.3%) (P<0.05).Conclusion AIH emerges as the most prevalent hepatic complication among PBC patients, whereas SS stands out as the dominant extrahepatic complication. When PBC becomes complicated with ATD, it poses a detrimental impact on patients' clinical prognosis.    

Key words: Primary biliary cholangitis, Autoimmune diseases, Autoimmune hepatitis, Sjogren's syndrome