Hatami Behzad, Pasharavesh Leila, Sharifian Afsaneh, Zali Mohammad Reza
Gastroenterology and Liver Diseases Research Center, Research Institute for Gastroenterology and Liver Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Basic and Molecular Epidemiology of Gastrointestinal Disorders Research Center, Research Institute for Gastroenterology and Liver Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Gastroenterol Hepatol Bed Bench. 2023;16(3):259-269. doi: 10.22037/ghfbb.v16i2.2589.
Primary Sclerosing Cholangitis (PSC) is a chronic cholestatic liver disease which is associated with Inflammatory Bowel Disease (IBD) in 70% of cases. It seems PSC/IBD is a distinct phenotype that is different from PSC, and IBD alone. Hence, we review the epidemiology, pathogenesis, natural course and management of PSC/IBD before and after LT for PSC. Extensive colitis, rectal sparing, backwash ileitis, and mild symptoms are the characteristics of IBD coexisting with PSC. Moreover, PSC patients with concurrent IBD have higher risk of cholangiocarcinoma, and colorectal neoplasia predominantly in right colon and at younger age. Therefore, it is essential to monitor these individuals continuously. It is interesting to note that the course of IBD (ulcerative colitis) after liver transplantation (LT) for PSC varies greatly, and some patients may develop worsening colitis after LT despite immunosuppressive regimens. As well, management of these patients was discussed in this review.
原发性硬化性胆管炎（PSC）是一种慢性胆汁淤积性肝病，70%的病例与炎症性肠病（IBD）相关。PSC/IBD似乎是一种不同于单纯PSC和单纯IBD的独特表型。因此，我们回顾了PSC患者肝移植前后PSC/IBD的流行病学、发病机制、自然病程及管理。广泛结肠炎、直肠 spared、反流性回肠炎和轻微症状是与PSC并存的IBD的特征。此外，合并IBD的PSC患者患胆管癌和结直肠肿瘤的风险更高，主要发生在右半结肠且发病年龄较轻。因此，持续监测这些个体至关重要。值得注意的是，PSC患者肝移植后IBD（溃疡性结肠炎）的病程差异很大，尽管有免疫抑制方案，一些患者在肝移植后仍可能出现结肠炎恶化。同样，本综述也讨论了这些患者的管理。
