Medford Abigail, Childs Jonathan, Little Ashleigh, Chakraborty Sanjukta, Baiocchi Leonardo, Alpini Gianfranco, Glaser Shannon
Department of Medical Physiology, Texas A&M University School of Medicine, Bryan, TX, USA.
Hepatology Unit, University of Tor Vergata, Rome, Italy.
J Clin Transl Hepatol. 2023 Aug 28;11(4):949-957. doi: 10.14218/JCTH.2022.00398. Epub 2023 Mar 16.
The liver has a vital role in many metabolic and regulatory processes in the body. Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is a chronic cholestatic autoimmune disease of the intrahepatic bile ducts associated with loss of tolerance to mitochondrial antigens. At this time there is no definitive cure for PBC; however, ursodeoxycholic acid (UDCA) has been shown to reduce injury when administered as the first line of treatment. Additional therapeutics can be given concurrently or as an alternative to UDCA to manage the symptoms and further curb disease progression. Currently, a liver transplant is the only potentially curative option when the patient has developed end-stage liver disease or intractable pruritus. This review aims to delineate the pathogenesis of primary biliary cholangitis and shed light on current therapeutic strategies in the treatment of PBC.
肝脏在人体许多代谢和调节过程中起着至关重要的作用。原发性胆汁性胆管炎(PBC),以前称为原发性胆汁性肝硬化,是一种肝内胆管的慢性胆汁淤积性自身免疫性疾病,与对线粒体抗原的耐受性丧失有关。目前,PBC尚无确切的治愈方法;然而,熊去氧胆酸(UDCA)作为一线治疗药物已被证明可减轻损伤。可以同时给予其他治疗药物或作为UDCA的替代药物来控制症状并进一步遏制疾病进展。目前,当患者发展为终末期肝病或顽固性瘙痒时,肝移植是唯一可能治愈的选择。本综述旨在阐述原发性胆汁性胆管炎的发病机制,并阐明目前治疗PBC的治疗策略。