Mandea Matei, Oancea Dragos M, Ghioca Mihaela C, Iacob Speranta M, Iacob Razvan A, Lupescu Ioana G, Gheorghe Liliana S
Department of Internal Medicine, Discipline of Gastroenterology and Hepatology, University of Medicine and Pharmacy Carol Davila, 020021 Bucharest, Romania.
Digestive Diseases and Liver Transplant Center, Fundeni Clinical Institute, 022328 Bucharest, Romania.
J Clin Med. 2025 Aug 21;14(16):5915. doi: 10.3390/jcm14165915.
This retrospective longitudinal study evaluated the significance of cholestasis syndrome and the diagnosis of primary sclerosing cholangitis (PSC) in inflammatory bowel disease (IBD) patients from a tertiary center in Romania. From 2011 to 2022, 3767 patients suspected for IBD were evaluated, with 2499 confirmed cases. Of these, 34 patients (1.36%) had an IBD-PSC phenotype. Of the IBD-PSC cases, 56% were associated with UC and 44% with CD. Enzymatic cholestasis was observed in 13.3% of IBD patients, with gamma-glutamyl transpeptidase (GGT) elevated in 70.2% and alkaline phosphatase (ALP) in 51.3%. However, only 10.2% of the patients with enzymatic cholestasis were diagnosed with PSC. Other liver diseases identified included metabolic-associated steatotic liver disease (MASLD), chronic viral hepatitis, Primary Biliary Cholangitis, autoimmune hepatitis, and liver neoplasms. A higher incidence of cholangiocarcinoma (11.76% vs. 0.24%, < 0.001) and liver-related death (8.82% vs. 0.65%, < 0.001) was found between IBD-PSC patients and those without PSC. PSC-CD patients were diagnosed at a younger age (30.2 vs. 43 years, < 0.001), had higher rates of severe disease (73.3% vs. 10.5%, < 0.001), required more biological treatment (60% vs. 15.7%, < 0.001), and experienced higher mortality (20% vs. 0%, < 0.001). This study represents the most extensive cohort analysis of PSC-IBD patients in Romania and Eastern Europe, highlighting clinical differences between PSC-UC and PSC-CD phenotypes. The regular monitoring of ALP and GGT in IBD patients helps detect liver diseases, including PSC. However, only one in ten patients with IBD and enzymatic cholestasis was diagnosed with PSC.
这项回顾性纵向研究评估了罗马尼亚一家三级中心炎症性肠病(IBD)患者胆汁淤积综合征的意义及原发性硬化性胆管炎(PSC)的诊断情况。2011年至2022年期间,对3767例疑似IBD患者进行了评估,确诊2499例。其中,34例患者(1.36%)具有IBD-PSC表型。在IBD-PSC病例中,56%与溃疡性结肠炎(UC)相关,44%与克罗恩病(CD)相关。13.3%的IBD患者出现酶性胆汁淤积,70.2%的患者γ-谷氨酰转肽酶(GGT)升高,51.3%的患者碱性磷酸酶(ALP)升高。然而,仅有10.2%的酶性胆汁淤积患者被诊断为PSC。鉴定出的其他肝脏疾病包括代谢相关脂肪性肝病(MASLD)、慢性病毒性肝炎、原发性胆汁性胆管炎、自身免疫性肝炎和肝脏肿瘤。IBD-PSC患者与无PSC患者相比,胆管癌发病率更高(11.76%对0.24%,<0.001),肝脏相关死亡率更高(8.82%对0.65%,<0.001)。PSC-CD患者诊断时年龄更小(30.2岁对43岁,<0.001),重症疾病发生率更高(73.3%对10.5%,<0.001),需要更多生物治疗(60%对15.7%,<0.001),死亡率更高(20%对0%,<0.001)。本研究是罗马尼亚和东欧对PSC-IBD患者最广泛的队列分析,突出了PSC-UC和PSC-CD表型之间的临床差异。对IBD患者定期监测ALP和GGT有助于发现包括PSC在内的肝脏疾病。然而,IBD合并酶性胆汁淤积的患者中,每十人中只有一人被诊断为PSC。