WS16.3 Consecutive transient elastography measurements to detect cystic fibrosis liver disease

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Background Cystic fibrosis (CF) related liver disease (CFLD) is diagnosed using a combination of criteria. Transient elastography (TE) (ultrasonographic method evaluating liver stiffness) differentiates CF patients with and without liver disease (CFnoLD) and identifies patients with an increased risk for portal hypertension. Aim: Detect evolving CFLD using TE measurements. Method Retrospective study (2007–2013) including all patients with TE measurements, performed by the same operator. Measurement was correlated to the presence or development of CFLD based on the medical files. Results 150 CF patients [median age 17 (9–24) years] were included, 118 with repeated TE: 20 (14%) had CFLD at the first TE measurement, 4 (3%) developed CFLD during follow-up. The median TE value in CFLD was 14 (8.7–32.2) compared to 5.3 (4.9–5.7) in CFnoLD (P = 0.0001). The intra-individual differences between 2 consecutive measurements [median interval between measurements 1 yr (1–2)] was 0.05 (–1, 1.2) in CFnoLD and 0.55 (–1.68, 1.53) in the CFLD patients. The area under the receiver operating curve for TE predicting CFLD was 0.985. TE measurements above 6.55 kPa predicted CFLD with a sensitivity of 94.7% and a specificity of 90.8% according to the AUROC. In CF Conclusion TE measurements progressively increased in CF patients developing CFLD. A prospective study is needed to evaluate whether TE will be able to detect CFLD before it becomes clinically apparent.