Zhang Wentao, Xu Bing, Ren Danfeng, Zhao Yingren, Liu Jinfeng
Department of Infectious Diseases, The First Affiliated Hospital of Xi'an Jiaotong University, Xian, CHN.
Cureus. 2025 Aug 12;17(8):e89942. doi: 10.7759/cureus.89942. eCollection 2025 Aug.
Despite the significant advances in autoimmune research, primary biliary cholangitis (PBC) remains a clinically challenging and incompletely understood disease, with its exact causes and molecular mechanisms still elusive. The immune system exerts crucial pathogenic effects throughout PBC development, from initial biliary injury to end-stage cirrhosis. Despite decades of investigation, the current understanding of PBC pathogenesis remains fragmented, particularly regarding the immune mechanisms involved in the disease. To address this, we conducted a bibliometric analysis of immunological studies on PBC, utilizing data from the Web of Science database, which covers publications from 1970 to 2023, and identified 2,042 studies for analysis. The findings revealed a steady increase in research activity over the decades. Our analysis revealed a consistent upward trend in PBC-related studies, with an average growth rate of 14.45%. The United States leads as the top contributing country, with the University of California, Davis, and Dr. Gershwin M. Eric emerging as the most influential institution and author, respectively. Among journals, Hepatology is the most prominent, with the highest number of citations and co-citations. Recent advances in diagnostic techniques have substantially improved early detection rates of PBC while dramatically decreasing instances of disease decompensation. This paradigm shift has redirected research priorities toward innovative disease management strategies and patient-centered care approaches. This synthesis presents a unified framework for PBC immunopathogenesis, pinpointing critical knowledge gaps and high-potential research trajectories in disease mechanisms and therapeutic innovation.
尽管自身免疫研究取得了重大进展,但原发性胆汁性胆管炎(PBC)仍然是一种临床上面临挑战且尚未完全理解的疾病,其确切病因和分子机制仍然难以捉摸。在PBC的整个发展过程中,从最初的胆管损伤到终末期肝硬化,免疫系统都发挥着关键的致病作用。尽管经过了数十年的研究,但目前对PBC发病机制的理解仍然支离破碎,尤其是关于该疾病所涉及的免疫机制。为了解决这个问题,我们利用科学网数据库的数据,对PBC的免疫学研究进行了文献计量分析,该数据库涵盖了1970年至2023年的出版物,并确定了2042项研究进行分析。研究结果显示,几十年来研究活动稳步增加。我们的分析显示,与PBC相关的研究呈持续上升趋势,平均增长率为14.45%。美国是贡献最大的国家,加利福尼亚大学戴维斯分校和格什温·M·埃里克博士分别成为最具影响力的机构和作者。在期刊方面,《肝脏病学》最为突出,被引次数和共被引次数最多。诊断技术的最新进展大幅提高了PBC的早期检测率,同时显著减少了疾病失代偿的情况。这种范式转变将研究重点转向了创新的疾病管理策略和以患者为中心的护理方法。本综述为PBC免疫发病机制提供了一个统一的框架,指出了疾病机制和治疗创新方面的关键知识空白和高潜力研究轨迹。