RESUMEN
BACKGROUND AND OBJECTIVE: Reliable markers are needed for early diagnosis and follow-up of liver disease in Cystic Fibrosis (CF). The objective was to evaluate the diagnostic performance of Transient Elastography (TE), Real-Time ShearWave Ultrasound Elastography (SWE), Magnetic Resonance Elastography (MRE) and the FibroTest as markers of Cystic Fibrosis Liver Disease (CFLD). METHODS: A monocentric prospective cross-modality comparison study was proposed to all children (6 to 18 years of age) attending the CF center. Based on liver ultrasound findings, participants were classified into 3 groups: multinodular liver or portal hypertension (Nodular US/PH, advanced CFLD), heterogeneous increased echogenicity (Heterogeneous US, CFLD) or neither (Normal/Homogeneous US, no CFLD). The 4 tests were performed on the same day. The primary outcome was the FibroTest value and liver stiffness measurements (LSM). RESULTS: 55 participants (mean age 12.6 ± 3.3 years; 25 girls) were included between 2015 and 2018: 23 in group Nodular US/PH, 8 in group Heterogeneous US and 24 in group Normal/Homogeneous US (including 4 with steatosis). LSM on TE, SWE and MRE were higher in participants with CFLD (groups Nodular US/PH and Heterogeneous US) compared to others (group Normal/Homogeneous US) (p<0.01), while FibroTest values did not differ (p = 0.09). The optimal cut-off values for predicting CFLD on TE, SWE and MRE were 8.7 (AUC=0.83, Se=0.71, Sp=0.96), 7.8 (AUC=0.85, Se=0.73, Sp=0.96) and 4.15 kPa (AUC=0.68, Se=0.73, Sp=0.64), respectively. LSM predicted the occurrence of major liver-related events at 3 years. TE and SWE were highly correlated (Spearman's ρ=0.9) and concordant in identifying advanced CFLD (Cohen's κ=0.84) while MRE was moderately correlated and concordant with TE (ρ=0.41; κ=36) and SWE (ρ=0.5; κ=0.50). CONCLUSION: This study demonstrated excellent diagnostic performance of TE, SWE and MRE for the diagnosis of CFLD.