Ahn Dong-Won
Department of Internal Medicine, Seoul National University Boramae Medical Center, Seoul National University College of Medicine, Seoul, Korea.
Korean J Gastroenterol. 2020 May 25;75(5):246-256. doi: 10.4166/kjg.2020.75.5.246.
Primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC) are immune-mediated chronic liver diseases. PSC is a rare disorder characterized by multi-focal bile duct strictures and progressive liver diseases that ultimately results in the need for liver transplantation in most patients. Imaging studies, such as MRCP, have an essential role in the diagnosis of most cases of PSC. PSC is usually accompanied by inflammatory bowel disease, and there is a high risk of cholangiocarcinoma and colorectal cancer in PSC. No medical therapies have been proven to delay the progression of PSC. Endoscopic intervention for tissue diagnosis or biliary drainage is frequently required in cases of PSC with a dominant stricture, acute cholangitis, or clinically suspected cholangiocarcinoma. PBC is a chronic inflammatory autoimmune cholestatic liver disease, which, when untreated, will culminate in end-stage biliary cirrhosis requiring liver transplantation. A diagnosis is usually based on the presence of serum liver tests indicative of cholestatic hepatitis in association with circulating antimitochondrial antibodies. Patient presentation and course can be diverse in PBC, and risk stratification is important for ensuring that all patients receive a personalized approach to their care. Medical therapy using ursodeoxycholic acid or obeticholic acid has an important role in reducing the progression to end-stage liver disease in PBC.
原发性硬化性胆管炎(PSC)和原发性胆汁性胆管炎(PBC)是免疫介导的慢性肝病。PSC是一种罕见疾病,其特征为多灶性胆管狭窄和进行性肝病,最终大多数患者需要进行肝移植。影像学检查,如磁共振胰胆管造影(MRCP),在大多数PSC病例的诊断中起着至关重要的作用。PSC通常伴有炎症性肠病,且PSC患者患胆管癌和结直肠癌的风险较高。尚无医学疗法被证实可延缓PSC的进展。对于存在显性狭窄、急性胆管炎或临床怀疑胆管癌的PSC病例,经常需要进行内镜干预以进行组织诊断或胆道引流。PBC是一种慢性炎症性自身免疫性胆汁淤积性肝病,若不治疗,最终将发展为终末期胆汁性肝硬化而需要肝移植。诊断通常基于血清肝功能检查结果提示胆汁淤积性肝炎并伴有循环抗线粒体抗体。PBC患者的临床表现和病程可能多种多样,风险分层对于确保所有患者都能接受个性化治疗至关重要。使用熊去氧胆酸或奥贝胆酸进行药物治疗在降低PBC进展至终末期肝病方面具有重要作用。