Department of Gastroenterology, Cliniques Universitaires Saint-Luc, Brussels, Belgium.
Department of Pneumology, Cliniques Universitaires Saint-Luc, Brussels, Belgium; Cystic Fibrosis Reference Center, Cliniques Universitaires Saint-Luc, Brussels, Belgium.
Clin Res Hepatol Gastroenterol. 2022 Nov;46(9):101977. doi: 10.1016/j.clinre.2022.101977. Epub 2022 Jun 27.
Cystic fibrosis liver disease (CFLD) is the third leading cause of death in patients with cystic fibrosis (CF). We aim to determine the prevalence of CFLD in a cohort of adult patients with CF and to characterise liver involvement in this population highlighting the importance of histological diagnosis.
We retrospectively studied a cohort of patients with CF. Inclusion criteria were age ≥ 18 and minimum 1 year of follow-up. We excluded lung transplant patients. CFLD was defined as having 2 out of 3 criteria: persistent elevation of transaminases and/or gamma-glutamyltransferase; abnormal ultrasound; and abnormal transient elastography. Non-invasive fibrosis biomarkers were calculated in CFLD patients. Adult-onset CFLD (Ad-CFLD) was defined as CFLD ≥18 years. Severe CFLD (s-CFLD) was defined as CFLD with cirrhosis and/or portal hypertension.
We included 113 patients. Median age was 29 years, 58 were male. Forty patients had CFLD. Median age at CFLD diagnosis was 10 years. Twenty-one patients had s-CFLD. Two s-CFLD patients had nodular regenerative hyperplasia, 1 had hepatocellular carcinoma and 4 underwent liver transplantation. Six patients had ad-CFLD. Both CFLD and s-CFLD groups were compared to a non-CFLD group. The CFLD group had significantly more males (p = 0.034). S-CFLD group had worse pulmonary function (p = 0.015).
Thirty five percent of adult patients with CF, mainly males, had CFLD. Nineteen percent had s-CFLD and had worse pulmonary function. With recent reports unravelling different pathophysiological mechanisms in CFLD, we believe it is important to better characterise liver involvement using liver biopsy.
囊性纤维化肝病（CFLD）是囊性纤维化（CF）患者的第三大死亡原因。我们旨在确定 CF 成年患者队列中 CFLD 的患病率，并描述该人群的肝脏受累情况，强调组织学诊断的重要性。
我们回顾性研究了一组 CF 患者。纳入标准为年龄≥18 岁且随访时间至少 1 年。我们排除了肺移植患者。CFLD 的定义为符合以下 3 项标准中的 2 项：转氨酶和/或γ-谷氨酰转移酶持续升高；超声异常；和异常的瞬时弹性成像。在 CFLD 患者中计算了非侵入性纤维化生物标志物。成人起病的 CFLD（Ad-CFLD）定义为 CFLD 年龄≥18 岁。严重的 CFLD（s-CFLD）定义为 CFLD 合并肝硬化和/或门静脉高压。
我们纳入了 113 名患者。中位年龄为 29 岁，58 名男性。40 名患者患有 CFLD。CFLD 诊断的中位年龄为 10 岁。21 名患者患有 s-CFLD。2 名 s-CFLD 患者患有结节性再生性增生，1 名患有肝细胞癌，4 名患者接受了肝移植。6 名患者患有 Ad-CFLD。将 CFLD 组和 s-CFLD 组与非 CFLD 组进行比较。CFLD 组男性明显更多（p=0.034）。s-CFLD 组的肺功能更差（p=0.015）。
35%的 CF 成年患者，主要是男性，患有 CFLD。19%患有 s-CFLD，且肺功能更差。鉴于最近的报告揭示了 CFLD 中不同的病理生理机制，我们认为使用肝活检更好地描述肝脏受累情况很重要。
