Liver Institute Northwest, , 3216 Northeast 45th Place, Suite 212, Seattle, WA 98105, USA.
Avera McKennan Hospital and University Health Center, Sioux Falls, SD, USA.
Clin Liver Dis. 2024 Feb;28(1):129-141. doi: 10.1016/j.cld.2023.07.005. Epub 2023 Aug 21.
Primary sclerosing cholangitis (PSC) is considered an immunologically mediated disease. However, some of its features are not consistent with the typical profile of autoimmune conditions. PSC is characterized by progressive biliary fibrosis that may ultimately result in the eventual development of cirrhosis. In recent years, multiple studies have reported that the incidence and prevalence of this disease are on the rise. Consequently, patients are often diagnosed without symptoms or signs of advanced liver disease, although many still present with signs of decompensated liver disease. This article discusses the epidemiology, clinical presentation, and diagnostic workup in patients with PSC.
原发性硬化性胆管炎（PSC）被认为是一种免疫介导的疾病。然而，其某些特征与自身免疫性疾病的典型特征并不相符。PSC 的特征是进行性胆管纤维化，最终可能导致肝硬化的发生。近年来，多项研究报告称，这种疾病的发病率和患病率呈上升趋势。因此，尽管许多患者仍存在失代偿性肝病的迹象，但患者通常在没有晚期肝病症状或体征的情况下被诊断出来。本文讨论了 PSC 患者的流行病学、临床表现和诊断方法。
