Abstract

PurposeTo identify independent imaging features and establish a diagnostic algorithm for diagnosis of cystic fibrosis (CF)-associated liver disease (CFLD) in CF patients compared to controls using gadoxetic acid-enhanced MRI.MethodsA total of 90 adult patients were enrolled: 50 with CF, 40 controls. The CF group was composed of two subgroups: a retrospective test subgroup (n = 33) and a prospective validation subgroup (n = 17). Controls (patients with normal liver enzymes and only benign focal liver lesions) were divided accordingly (27:13). MRI variables, including quantitative and qualitative parameters, were used to distinguish CFLD from controls using clinical symptoms, laboratory tests and Debray criteria. Disease severity was classified according to Child-Pugh and Albumin-Bilirubin (ALBI) scores. Fifteen qualitative single-lesion CF descriptors were defined. Two readers independently evaluated the images. Univariate statistical analysis was performed to obtain significant imaging features that differentiate CF patients from controls. Through multivariate analysis using chi-squared automatic interaction detector (CHAID) methodology the most important descriptors were identified. Diagnostic performance was assessed by receiver-operating characteristic (ROC) analysis.ResultsThree independent imaging descriptors distinguished CFLD from controls: (1) presence of altered gallbladder morphology; (2) periportal tracking; and (3) periportal fat deposition. Prospective validation of the classification algorithm demonstrated a sensitivity of 94.1% and specificity of 84.6% for discriminating CFLD from controls. Disease severity was well associated with the imaging features.ConclusionsA short unenhanced MRI protocol can identify the three cardinal imaging features of CFLD. The hepatobiliary phase of gadoxetic acid-enhanced MRI can define CFLD progression.Key Points• Using a multivariate classification analysis, we identified three independent imaging features, altered gallbladder morphology (GBAM), periportal tracking (PPT) and periportal fat deposition (PPFD), that could diagnose CFLD with high sensitivity, 94.1 % (95% CI: 71.3–99.9) and moderate specificity, 84.6 % (95% CI: 54.6–98.1).• Based upon the results of this study, gadoxetic acid-enhanced MRI with DWI is able to diagnose early-stage CFLD, as well as its progression.

Highlights

  • Cystic fibrosis (CF) is one of the most common, lethal, autosomal recessive diseases of the Caucasian population

  • Using a multivariate classification analysis, we identified three independent imaging features, altered gallbladder morphology (GBAM), periportal tracking (PPT) and periportal fat deposition (PPFD), that could diagnose cystic fibrosis-associated liver disease (CFLD) with high sensitivity, 94.1 % and moderate specificity, 84.6 %

  • Based upon the results of this study, gadoxetic acid-enhanced magnetic resonance imaging (MRI) with diffusion-weighted imaging (DWI) is able to diagnose early-stage CFLD, as well as its progression

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Summary

Introduction

Cystic fibrosis (CF) is one of the most common, lethal, autosomal recessive diseases of the Caucasian population. -called cystic fibrosis-associated liver disease (CFLD) may progress to end-stage liver cirrhosis, requiring curative liver transplantation [2]. Early diagnosis of CFLD is crucial as some centres may initiate UDCA treatment as early as possible to improve liver function [7]. Both clinical and biochemical findings have low sensitivity and specificity for CFLD [8]. Gadoxetic acid-enhanced MRI detects focal liver lesions and provides information about regional and global function of the hepatobiliary system [13]. DWI, because of its inherent ability to detect subtle changes in hepatobiliary tissue [14], would be expected to detect CFLD at very early stages

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