Chen Yangfan, Chen Ruofei, Li Haiyan, Shuai Zongwen
Department of Rheumatology and Immunology, The First Affiliated Hospital of Anhui Medical University, Hefei, 230022, China.
Inflammation and Immune Mediated Diseases Laboratory of Anhui Province, Hefei, 230032, China.
Immunol Res. 2025 Apr 7;73(1):67. doi: 10.1007/s12026-025-09622-9.
The three major autoimmune liver diseases are autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC).These conditions are assumed to result from a breakdown in immunological tolerance, which leads to an inflammatory process that causes liver damage.The self-attack is started by T-helper cell-mediated identification of liver autoantigens and B-cell production of autoantibodies,and it is maintained by a reduction in the number and activity of regulatory T-cells.Infections and environmental factors have been explored as triggering factors for these conditions, in addition to a genetic predisposition.Allelic mutations in the HLA locus have been linked to vulnerability, as have relationships with single nucleotide polymorphisms in non-HLA genes.Despite the advances in the management of these diseases, there is no curative treatment for these disorders, and a significant number of patients eventually progress to an end-stage liver disease requiring liver transplantation.In this line, tailored immune-therapeutics have emerged as possible treatments to control the disease.In addition, early diagnosis and treatment are pivotal for reducing the long-lasting effects of these conditions and their burden on quality of life.Herein we present a review of the etiology, clinical presentation, diagnosis, and challenges on ALDs and the feasible solutions for these complex diseases.
三种主要的自身免疫性肝病是自身免疫性肝炎(AIH)、原发性胆汁性胆管炎(PBC)和原发性硬化性胆管炎(PSC)。这些病症被认为是由免疫耐受的破坏引起的,这会导致炎症过程,进而造成肝损伤。自身攻击由辅助性T细胞介导的肝脏自身抗原识别和B细胞产生自身抗体引发,并通过调节性T细胞数量和活性的减少得以维持。除了遗传易感性外,感染和环境因素也被探讨为这些病症的触发因素。HLA基因座中的等位基因突变与易感性有关,非HLA基因中的单核苷酸多态性也与之相关。尽管这些疾病的治疗取得了进展,但尚无治愈性疗法,并且大量患者最终会进展为终末期肝病,需要进行肝移植。就此而言,量身定制的免疫疗法已成为控制该疾病的可能治疗方法。此外,早期诊断和治疗对于减轻这些病症的长期影响及其对生活质量的负担至关重要。在此,我们对自身免疫性肝病的病因、临床表现、诊断及挑战以及这些复杂疾病的可行解决方案进行综述。