EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis
EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis
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DOI：
10.1016/j.jhep.2017.03.022
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发表时间：
2017-07-01
影响因子：
25.7
通讯作者：
Marzioni, Marco
中科院分区：
文献类型：
--
作者：
Hirschfield, Gideon M.;Beuers, Ulrich;Marzioni, Marco
原发性胆汁性胆管炎（PBC）是一种慢性炎症性自身免疫性胆汁淤积性肝病，如果不及时治疗，最终会导致终末期胆汁性肝硬化。诊断通常基于血清肝脏检查是否存在，表明存在与循环抗线粒体抗体相关的胆汁淤积性肝炎。患者的表现和病程可能多种多样，风险分层对于确保所有患者接受个性化的护理方法非常重要。治疗和管理的目标是预防终末期肝病并改善相关症状。在实践中，为了减少疾病进展性的影响，目前的药理学方法包括许可疗法（熊去氧胆酸和奥贝胆酸）和标签外疗法（纤维酸衍生物、布地奈德）。这些临床实践指南总结了结构化、终身和个体化的 PBC 患者护理方法重要性的证据，提供了帮助临床医生诊断和有效管理患者的框架。 (C) 2017 年欧洲肝脏研究协会。由 Elsevier B.V. 出版。保留所有权利。
Primary biliary cholangitis (PBC) is a chronic inflammatory autoimmune cholestatic liver disease, which when untreated will culminate in end-stage biliary cirrhosis. Diagnosis is usually based on the presence of serum liver tests indicative of a cholestatic hepatitis in association with circulating antimitochondrial antibodies. Patient presentation and course can be diverse and risk stratification is important to ensure all patients receive a personalised approach to their care. The goals of treatment and management are the prevention of end-stage liver disease, and the amelioration of associated symptoms. Pharmacologic approaches in practice, to reduce the impact of the progressive nature of disease, currently include licensed therapies (ursodeoxycholic acid and obeticholic acid) and off-label therapies (fibric acid derivatives, budesonide). These clinical practice guidelines summarise the evidence for the importance of a structured, life-long and individualised, approach to the care of patients with PBC, providing a framework to help clinicians diagnose and effectively manage patients. (C) 2017 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.