Sheila Sherlock Liver Centre and UCL Institute for Liver and Digestive Health, University College London & Royal Free London NHS Foundation Trust, London, UK.
Department of Clinical and Experimental Medicine, Division of Clinical and Molecular Hepatology, University of Messina, Messina, Italy.
Eur J Clin Invest. 2019 May;49(5):e13088. doi: 10.1111/eci.13088. Epub 2019 Mar 18.
Cholangiocarcinoma (CCA) complicates primary sclerosing cholangitis (PSC) in 10%-20% of cases, but current tools for prediction of a CCA diagnosis are inadequate. Recently, we demonstrated the utility of the enhanced liver fibrosis (ELF) test to stratify prognosis in PSC. We observed that patients with PSC + CCA had significantly higher ELF score than those with PSC alone. In this study, we aimed to investigate further this association in a larger cohort of PSC patients with CCA compared with patients with PSC or CCA alone.
Stored sera from patients with PSC (n = 119), CCA without known chronic liver disease (n = 36) and PSC + CCA (n = 32) were tested for ELF. ELF score, gender, age, age at disease diagnosis, inflammatory bowel disease, PSC duration and severity, and CCA features were compared amongst the three cohorts. Factors related to an elevated ELF score were investigated.
Enhanced liver fibrosis score was significantly higher in patients with CCA without underlying chronic liver disease and in patients with PSC + CCA compared to those with PSC alone (P < 0.001). In multivariate analysis, elevated ELF score was associated with the diagnosis of CCA independently of age and PSC status (P < 0.001).
Enhanced liver fibrosis score was elevated in patients with CCA irrespective of the presence of PSC, and independently of liver disease stage. Our results indicate that the association between high ELF score and CCA may be related to the tumour's desmoplastic nature, independent of background liver fibrosis, suggesting that ELF score could be used to risk stratify for CCA in PSC.
胆管癌（CCA）在 10%-20%的原发性硬化性胆管炎（PSC）病例中并发，但目前用于预测 CCA 诊断的工具还不够完善。最近，我们证明了增强型肝纤维化（ELF）检测在 PSC 中的预后分层中的作用。我们观察到，PSC+C CA 患者的 ELF 评分明显高于单独 PSC 患者。在这项研究中，我们旨在更大的 PSC 患者队列中进一步研究这种与 CCA 的相关性，这些患者患有 CCA，而不是单独患有 PSC 或 CCA。
检测了 119 例 PSC 患者（n=119）、36 例无已知慢性肝病的 CCA 患者（n=36）和 32 例 PSC+C CA 患者（n=32）的储存血清中的 ELF。比较了三个队列的 ELF 评分、性别、年龄、疾病诊断年龄、炎症性肠病、PSC 持续时间和严重程度以及 CCA 特征。调查了与 ELF 评分升高相关的因素。
与单独患有 PSC 的患者相比，无潜在慢性肝病的 CCA 患者和 PSC+C CA 患者的增强型肝纤维化评分明显升高（P<0.001）。在多变量分析中，ELF 评分升高与 CCA 的诊断独立于年龄和 PSC 状态相关（P<0.001）。
无论是否存在 PSC，CCA 患者的 ELF 评分均升高，且独立于肝病分期。我们的结果表明，高 ELF 评分与 CCA 之间的关联可能与肿瘤的纤维增生性质有关，而与背景肝纤维化无关，这表明 ELF 评分可用于 PSC 中 CCA 的风险分层。
