Faculty of Medicine, Imperial College London, UK.
Department of Gastroenterology, St. Mark's Hospital, London, UK.
Br J Hosp Med (Lond). 2021 Nov 2;82(11):1-9. doi: 10.12968/hmed.2021.0247. Epub 2021 Nov 23.
Primary biliary cholangitis is a chronic condition characterised by autoimmune destruction of intralobular bile ducts. Publications have shown widespread gaps in the care of patients with primary biliary cholangitis. This article reviews the literature regarding currently licensed first- and second-line therapies and evaluates therapeutic options for symptomatic management of primary biliary cholangitis. Ursodeoxycholic acid is recommended for all patients with primary biliary cholangitis, with obeticholic acid available as second-line therapy, both having demonstrated safety and efficacy. Potential disease-modifying therapies, such as fibrates and budesonide, require further investigation before licensing. Cholestyramine is first-line therapy for pruritus, albeit with limited evidence and common side-effects. There is no licensed therapy for primary biliary cholangitis-related fatigue; treating underlying causes where applicable is recommended. Disease-modifying and symptomatic therapies must be considered in tandem when managing patients with primary biliary cholangitis. Emerging therapies show initial promise but further randomised trials with long-term follow up are required to evaluate their efficacy as single or combination therapies.
原发性胆汁性胆管炎是一种慢性疾病，其特征是小叶间胆管的自身免疫性破坏。研究表明，原发性胆汁性胆管炎患者的治疗存在广泛的差距。本文综述了关于目前批准的一线和二线治疗的文献，并评估了原发性胆汁性胆管炎症状管理的治疗选择。熊去氧胆酸推荐用于所有原发性胆汁性胆管炎患者，奥贝胆酸可用作二线治疗，两者均具有安全性和疗效。潜在的疾病修饰治疗，如贝特类药物和布地奈德，在获得许可之前需要进一步研究。考来烯胺是瘙痒的一线治疗药物，但证据有限，且常见副作用。原发性胆汁性胆管炎相关性疲劳尚无许可的治疗方法；建议在适用的情况下治疗潜在病因。在管理原发性胆汁性胆管炎患者时，必须同时考虑疾病修饰和症状治疗。新兴疗法初步显示出希望，但需要进行长期随访的随机对照试验，以评估其作为单一或联合治疗的疗效。
