Coman Diana, Bilodeau Marc, Vincent Catherine, Brochiero Emmanuelle, Lavoie Annick, Hercun Julian
Liver Unit, Centre Hospitalier de l'Université de Montréal, Montreal, Canada.
Laboratoire de Physiopathologie Pulmonaire, Centre de Recherche du Centre hospitalier de l'Université de Montréal (CRCHUM), Montreal, Canada.
Dig Dis Sci. 2025 Feb;70(2):526-532. doi: 10.1007/s10620-024-08784-0. Epub 2024 Dec 13.
The reported prevalence of cystic fibrosis (CF)-related liver disease (CFLD) reaches up to 40% in some cohorts. CFLD is the 3rd leading cause of mortality among patients with CF. The aims of this study were to evaluate the prevalence of CFLD in a cohort followed at a tertiary university center, to define the types of liver involvement, and to determine how non-invasive screening methods can be optimally integrated into clinical practice.
The files of patients followed at the CF clinic of the Centre hospitalier de l'Université de Montréal (CHUM) between 2020 and 2022 were retrospectively reviewed. The NIH criteria were used to define CFLD through the presence of one major criterion (abnormal imaging) or two minor criteria (persistently abnormal laboratory values, hepatosplenomegaly, or transient elastography (TE) ≥ 7 kPa).
A total of 357 patients were included in this study. CFLD was observed in 46 patients (13%). Among these, major criteria led to diagnosis in 43 patients (with or without minor criteria). TE performed best in non-invasive assessment of CLFD (area under the curve (AUROC) 0.80 (0.68-0.92, p = 0.0007)). A nodular liver was detected in 27 patients (7%), and was associated with higher non-invasive markers of fibrosis. In addition, presence of a nodular liver was associated with significant short-term mortality (14.8% vs. 1.5%, p = 0.003).
Early recognition of CFLD in clinical care can potentially prevent complications of cirrhosis and portal hypertension. The use of abdominal imaging and TE seems promising for detecting CFLD.
在某些队列研究中，报告的囊性纤维化（CF）相关肝病（CFLD）患病率高达40%。CFLD是CF患者中第三大死亡原因。本研究的目的是评估在一所三级大学中心随访的队列中CFLD的患病率，确定肝脏受累的类型，并确定如何将非侵入性筛查方法最佳地整合到临床实践中。
回顾性分析2020年至2022年在蒙特利尔大学中心医院（CHUM）CF诊所随访的患者档案。采用美国国立卫生研究院（NIH）标准，通过存在一项主要标准（影像学异常）或两项次要标准（实验室值持续异常、肝脾肿大或瞬时弹性成像（TE）≥7kPa）来定义CFLD。
本研究共纳入357例患者。46例患者（13%）观察到CFLD。其中，43例患者（有或无次要标准）通过主要标准确诊。TE在CLFD的非侵入性评估中表现最佳（曲线下面积（AUROC）为0.80（0.68-0.92，p=0.0007））。27例患者（7%）检测到肝脏结节，且与较高的纤维化非侵入性标志物相关。此外，肝脏结节的存在与显著的短期死亡率相关（14.8%对1.5%，p=0.003）。
在临床护理中早期识别CFLD可能预防肝硬化和门静脉高压的并发症。腹部影像学检查和TE的应用在检测CFLD方面似乎很有前景。
