Association between changes in LS and PROMs.

Background & aimsPrimary sclerosing cholangitis (PSC) is a rare chronic liver disease that impact quality of life (QoL). This study investigated the association between biomarkers of PSC disease severity and QoL.MethodsProspective study involving 80 participants with PSC at baseline and 55 at 1-year follow-up. QoL was assessed using patient reported outcomes (PROMs): RAND-SF36, SF6D and PSC-PRO. MRI-MRCP data was analysed using LiverMultiscan for iron-corrected liver T1 (cT1) and MRCP+ for the relative severity of intrahepatic biliary dilatations (RSIBD). Disease severity was also classified using FibroScan liver stiffness (LS), enhanced liver fibrosis (ELF), Mayo risk score (MRS), Amsterdam-Oxford model (AOM), alkaline phosphatase (ALP), presence of extrahepatic disease and dominant stricture (DS). Descriptive and regression analyses were conducted.ResultsAt baseline, more advanced PSC was associated with differences in PROMs, AOM > 2, (PSC-PRO PSC symptoms, 5.181, p = 0.048), LS > 9.6 kPa (SF-6D, −0.081, p = 0.027), cT1 > 825ms (SF6D QoL, −0.161, p = 0.004; SF36 PCS, −10.595, p = 0.001; SF36 MCS, −10.726, p = 0.012), DS (PSC-PRO symptoms scores, 5.800, p = 0.025), RSIBD (SF-6D, −0.081, p = 0.016). During follow-up, increase in LS was associated with a reduction in QoL measured via SF-6D (−0.002, p ConclusionsQoL in PSC was associated with biomarkers of parenchymal liver fibrosis (LS, cT1), biliary disease (dominant strictures, RSIBD), and composite scores of disease severity (AOM). Increasing LS predicted further declines in QoL. Further research should explore MRCP+ metrics and their impact on QoL.

背景与目的：原发性硬化性胆管炎（Primary sclerosing cholangitis, PSC）是一种罕见的慢性肝病，可显著影响患者的生活质量（Quality of Life, QoL）。本研究旨在探讨PSC疾病严重程度相关生物标志物与患者生活质量之间的关联。方法：本研究为前瞻性队列研究，共纳入80名基线期PSC患者，其中55名完成1年随访。采用患者报告结局指标（Patient Reported Outcomes, PROMs）评估生活质量，具体包括RAND-SF36、SF6D及PSC-PRO量表。通过LiverMultiscan分析MRI-MRCP影像数据，获取铁校正肝脏T1值（iron-corrected liver T1, cT1）；利用MRCP+工具计算肝内胆管扩张相对严重程度（Relative Severity of Intrahepatic Biliary Dilatations, RSIBD）。此外，本研究还采用FibroScan肝脏硬度值（Liver Stiffness, LS）、增强肝纤维化（Enhanced Liver Fibrosis, ELF）、Mayo风险评分（Mayo Risk Score, MRS）、阿姆斯特丹-牛津模型（Amsterdam-Oxford Model, AOM）、碱性磷酸酶（Alkaline Phosphatase, ALP）、肝外病变及显性狭窄（dominant stricture, DS）对疾病严重程度进行分级。本研究采用描述性分析与回归分析完成数据统计。结果：基线期时，疾病程度更严重的PSC患者多项PROMs指标出现显著差异：AOM＞2时（PSC-PRO症状评分：5.181，p=0.048）、LS＞9.6kPa时（SF-6D：-0.081，p=0.027）、cT1＞825ms时（SF6D生活质量评分：-0.161，p=0.004；SF36躯体健康总分（PCS）：-10.595，p=0.001；SF36精神健康总分（MCS）：-10.726，p=0.012）、存在DS（PSC-PRO症状评分：5.800，p=0.025）、RSIBD升高（SF-6D：-0.081，p=0.016）。随访期间，肝脏硬度值升高与SF-6D评估的生活质量下降相关（-0.002，p=）。结论：PSC患者的生活质量与肝实质纤维化生物标志物（LS、cT1）、胆道疾病相关指标（显性狭窄、RSIBD）及疾病严重程度综合评分（AOM）显著相关。肝脏硬度值升高可预测患者生活质量的进一步下降。未来研究应进一步探索MRCP+相关指标及其对生活质量的影响。