Objective To investigate the impact of anticentromere antibody （ACA） on the clinical features and prognosis of patients with primary biliary cholangitis （PBC） by comparing clinical classification，ursodeoxycholic acid （UDCA） response，GLOBE score，and UK-PBC score between ACA-positive PBC patients and ACA-negative PBC patients. Methods A total of 749 patients who were admitted to Beijing Ditan Hospital，Capital Medical University，from August 2013 to December 2022 and were diagnosed with PBC were enrolled and divided into ACA-positive group with 147 patients and ACA-negative group with 602 patients. According to their conditions on admission，the two groups were compared in terms of the distribution of clinical types，i.e.，chronic progression-type PBC，portal hypertension-type PBC，and standard jaundice/liver failure-type PBC. There were 261 patients with complete data after 1-year follow-up，among whom there were 53 patients with positive ACA and 208 with negative ACA. A statistical analysis was performed，and propensity score matching was performed based on sex and age at a ratio of 1∶2. The two groups were compared in terms of 1-year UDCA response rate，GLOBE score，and UK-PBC score before and after matching. The independent-samples t test or the Mann-Whitney U test was used for comparison of continuous data between two groups，and the chi-square test was used for comparison of categorical data between two groups. Results Compared with the ACA-negative group，the ACA-positive group had a significantly higher age （61.28±10.35 years vs 56.74±12.17 years，t=4.164，P<0.001），a significantly higher proportion of female patients （93.9% vs 77.6%，χ²=20.221，P<0.001），a significantly higher proportion of patients with portal hypertension （48.3% vs 27.6%，χ²=23.289，P<0.001），and a significantly lower proportion of patients with jaundice/liver failure （24.5% vs 38.5%，χ²=10.205，P<0.001）. After 1-year follow-up，for the 261 PBC patients with complete data，there was no significant difference in UDCA response rate before propensity score matching between the ACA-positive group and the ACA-negative group （41.5% vs 41.8%，P>0.05），and there was a significant difference in the proportion of patients with a GLOBE score of >0.3 between the ACA-positive group and the ACA-negative group （92.5% vs 80.3%，χ²=3.935，P=0.047）. There were 53 patients in the ACA-positive group and 106 patients in the ACA-negative group after propensity score matching，and there were no significant differences between the two groups in UDCA response rate，GLOBE score，and UK-PBC score （all P>0.05）. Conclusion ACA-positive patients tend to have an older age，with a higher proportion of female patients or patients with portal hypertension，while there is a relatively low proportion of patients with jaundice/liver failure. Positive ACA has no significant impact on UDCA response rate，GLOBE score，and UK-PBC score.