Konstantis Georgios, Moghadam Dorsa Ghaffar Loy, Frey Alexandra, Nuruzade Nargiz, Schramm Christoph, Gerges Christian, Lange Christian M, Schmidt Hartmut, Willuweit Katharina, Kahraman Alisan, Passenberg Moritz, Rashidi-Alavijeh Jassin
Universitätsklinikum Essen, Essen, Germany.
Department of Gastroenterology, Hepatology and Transplant Medicine, University of Duisburg-Essen, Essen, Germany.
BMJ Open Gastroenterol. 2025 Jan 4;12(1):e001571. doi: 10.1136/bmjgast-2024-001571.
Secondary sclerosing cholangitis (SSC) represents a disease with a poor prognosis increasingly diagnosed in clinical settings. Notably, SSC in critically ill patients (SSC-CIP) is the most frequent cause. Variables associated with worse prognosis remain unclear. The primary aim of this study was to identify factors associated with transplant-free survival in SSC-CIP patients using readily available data.
A cohort of 47 patients diagnosed with SSC-CIP was retrospectively analysed for clinical, biochemical and endoscopic variables. Kaplan-Meier survival curves, log-rank tests and univariate Cox proportional hazards models were used to assess associations with transplant-free survival. A multivariable Cox regression model was constructed using Lasso regularisation and validated with Bootstrap resampling. Model performance was assessed using the C-statistic for discrimination.
Kaplan-Meier analysis identified bile duct obstruction requiring stent placement, and cholangitis episodes, as significant prognostic factors. In univariable analysis, age over 47 years (HR 2.61 (95% CI 1.02, 7.06), p=0.04), at least one cholangitis episode (HR 2.46 (95% CI 1.005, 6.06), p=0.04), stent placement (HR 2.89 (95% CI 1.13, 7.38), p=0.03), lower albumin levels (HR 0.52 (95% CI 0.28, 0.97), p=0.04) and higher international normalised ratio (INR) (HR 3.22 (95% CI 1.09, 9.53), p=0.03) were significant. Multivariable analysis showed that age at diagnosis, albumin and INR were significant independent predictors. The C-index was 0.78 (95% CI 0.65, 0.91), surpassing the model of end-stage liver disease score's prognostic accuracy (Concordance Index at 3 years: 66.2% vs 74.9%).
These findings provide valuable insights for establishing standard exception criteria for this rare liver disease, which could lead to improved organ allocation. Further prospective multicentre studies are necessary to validate our findings.
继发性硬化性胆管炎（SSC）是一种预后较差的疾病，在临床环境中诊断日益增多。值得注意的是，危重症患者中的SSC（SSC-CIP）是最常见的病因。与较差预后相关的变量仍不清楚。本研究的主要目的是利用现成的数据确定与SSC-CIP患者无移植生存相关的因素。
对47例诊断为SSC-CIP的患者队列进行回顾性分析，分析其临床、生化和内镜变量。采用Kaplan-Meier生存曲线、对数秩检验和单变量Cox比例风险模型评估与无移植生存的相关性。使用Lasso正则化构建多变量Cox回归模型，并通过Bootstrap重采样进行验证。使用C统计量评估模型性能以进行判别。
Kaplan-Meier分析确定需要放置支架的胆管梗阻和胆管炎发作是重要的预后因素。在单变量分析中，47岁以上（HR 2.61（95%CI 1.02，7.06），p=0.04）、至少一次胆管炎发作（HR 2.46（95%CI 1.005，6.06），p=0.04）、放置支架（HR 2.89（95%CI 1.13，7.38），p=0.03）、较低的白蛋白水平（HR 0.52（95%CI 0.28，0.97），p=0.04）和较高的国际标准化比值（INR）（HR 3.22（95%CI 1.09，9.53），p=0.03）具有显著性。多变量分析显示，诊断时的年龄、白蛋白和INR是显著的独立预测因素。C指数为0.78（95%CI 0.65，0.91），超过了终末期肝病评分模型的预后准确性（3年时的一致性指数：66.2%对74.9%）。
这些发现为建立这种罕见肝病的标准例外标准提供了有价值的见解，这可能会改善器官分配。需要进一步进行前瞻性多中心研究来验证我们的发现。
