Division of Internal Medicine, Department of Gastroenterology and Hepatology, University of Connecticut Health Center, Farmington, CT, USA.
Department of Pathology and Lab Medicine, University of Connecticut Health Center, Farmington, CT, USA.
J Clin Transl Hepatol. 2014 Dec;2(4):266-84. doi: 10.14218/JCTH.2014.00024. Epub 2014 Dec 15.
Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are two major types of chronic cholestatic liver disease. Each disorder has distinguishing features and variable progression, but both may ultimately result in cirrhosis and hepatic failure. The following offers a review of PBC and PSC, beginning with a general overview of disease etiology, pathogenesis, diagnosis, clinical features, natural course, and treatment. In addition to commonly associated manifestations of fatigue, pruritus, and fat-soluble vitamin deficiency, select disease-related topics pertaining to women's health are discussed including metabolic bone disease, hyperlipidemia and cardiovascular risk, and pregnancy-related issues influencing maternal disease course and birth outcomes. This comprehensive review of PBC and PSC highlights some unique clinical considerations in the care of female patients with cholestatic liver disease.
原发性胆汁性胆管炎 (PBC) 和原发性硬化性胆管炎 (PSC) 是两种主要的慢性胆汁淤积性肝病。每种疾病都有其独特的特征和不同的进展,但都可能最终导致肝硬化和肝衰竭。本文综述了 PBC 和 PSC,首先概述了疾病的病因、发病机制、诊断、临床特征、自然病程和治疗。除了常见的疲劳、瘙痒和脂溶性维生素缺乏相关表现外,还讨论了与女性健康相关的一些特定疾病相关话题,包括代谢性骨病、高脂血症和心血管风险,以及影响母体疾病过程和分娩结局的妊娠相关问题。对 PBC 和 PSC 的全面综述强调了在治疗胆汁淤积性肝病女性患者时需要考虑的一些独特的临床问题。