Shah Yash R, Nombera-Aznaran Natalia, Guevara-Lazo David, Calderon-Martinez Ernesto, Tiwari Angad, Kanumilli SriLakshmiDevi, Shah Purva, Pinnam Bhanu Siva Mohan, Ali Hassam, Dahiya Dushyant Singh
Department of Internal Medicine, Trinity Health Oakland, Pontiac, MI 48341, United States.
Faculty of Medicine, Universidad Peruana Cayetano Heredia, Lima 15102, Peru.
World J Hepatol. 2023 Aug 27;15(8):939-953. doi: 10.4254/wjh.v15.i8.939.
Primary sclerosing cholangitis (PSC) is a chronic and progressive immune-mediated cholangiopathy causing biliary tree inflammation and scarring, leading to liver cirrhosis and end-stage liver disease. Diagnosis of PSC is challenging due to its nonspecific symptoms and overlap with other liver diseases. Despite the rising incidence of PSC, there is no proven medical therapy that can alter the natural history of the disease. While liver transplantation (LT) is the most effective approach for managing advanced liver disease caused by PSC, post-transplantation recurrence of PSC remains a challenge. Therefore, ongoing research aims to develop better therapies for PSC, and continued efforts are necessary to improve outcomes for patients with PSC. This article provides an overview of PSC's pathogenesis, clinical presentation, and management options, including LT trends and future aspects. It also highlights the need for improved therapeutic options and ethical considerations in providing equitable access to LT for patients with PSC. Additionally, the impact of liver transplant on the quality of life and psychological outcomes of patients with PSC is discussed. Ongoing research into PSC's pathogenesis and post-transplant recurrence is crucial for improved understanding of the disease and more effective treatment options.
原发性硬化性胆管炎（PSC）是一种慢性进行性免疫介导的胆管病，可导致胆管树炎症和瘢痕形成，进而发展为肝硬化和终末期肝病。由于其症状不具特异性且与其他肝脏疾病存在重叠，PSC的诊断颇具挑战性。尽管PSC的发病率呈上升趋势，但尚无经证实的药物疗法能够改变该疾病的自然病程。虽然肝移植（LT）是治疗由PSC引起的晚期肝病最有效的方法，但PSC移植后复发仍是一个难题。因此，正在进行的研究旨在开发更好的PSC治疗方法，并且需要持续努力以改善PSC患者的治疗效果。本文概述了PSC的发病机制、临床表现及管理方案，包括肝移植趋势和未来展望。它还强调了在为PSC患者提供公平的肝移植机会方面，需要改进治疗选择并考虑伦理因素。此外，还讨论了肝移植对PSC患者生活质量和心理状况的影响。对PSC发病机制和移植后复发的持续研究对于更好地理解该疾病和开发更有效的治疗选择至关重要。
