Abstract
About 30% of patients with Cystic Fibrosis (CF) develop CF-associated liver disease (CFLD). Recent studies have shown that transient elastography (TE), as a method to quantify liver stiffness, allows non-invasive diagnosis of CFLD in adults and children with CF. Within this study we aimed to prospectively identify patients at risk for development of CFLD by longitudinal analysis of liver stiffness and fibrosis scores in a 5-year follow-up. 36 pediatric and 16 adult patients with initial liver stiffness below the cut-off value indicative of CFLD (6.3 kPa) were examined by transient elastography for 4–5 years. TE, APRI-, and FIB-4-scores were assessed and compared by Kruskal-Wallis test and receiver operating characteristic (ROC)-analysis. Frequencies were compared by Chi2-test. Among the 36 patients participating in this study, a subgroup of 9 patients developed liver stiffness >6.3 kPa after 4–5 years with an increase of ΔTE >0.38 kPa/a (the group with increasing liver stiffness was labelled TEinc). APRI- and FIB-4 scores confirmed the rationale for grouping. The frequency of CFLD assessed by conventional diagnosis was significantly higher in TEinc-group compared to the control group (TEnorm). None of the adult CF patients matched criteria for TEinc-group. For the first time it was shown that the non-invasive longitudinal assessment of TE allows identification of patients with progression of CFLD in a subgroup of juvenile but not in adult CF patients. Comparing TE to conventional fibrosis-scores underlined the strength of the continuous assessment of liver stiffness for the exact diagnosis of progressive CFLD. The newly described cut-off for pathologic increase of liver stiffness, ΔTEcutoff = 0.38kPa/a, might enable to detect developing CFLD using consequent follow up TE measurements before reaching the level of stiffness indicating established CFLD. Nevertheless, the limited size of the analyzed cohort should encourage a prospective, multi-center, long term follow up study to confirm the suggested cut-off for the rise in liver stiffness.
Highlights
Cystic fibrosis (CF) is the most common multisystem autosomal recessive genetic disorder with a high morbidity and mortality in Caucasian population with an incidence of approximately 1 of 3500 births worldwide [1]
Our results show that this approach has the potential to identify CF patients at risk of developing progression of CF liver disease
For 60 of the 173 CF patients, including 16 adults and 44 children, follow-up data with initial and regularly consecutive transient elastography (TE) over 4–5 years and quantification of APRI- and FIB-4 scores were available (Fig 1). 21 of the 44 pediatric patients were diagnosed with Cystic Fibrosis associated liver disease (CFLD) as defined by clinical guidelines criteria [8] during the follow-up
Summary
Cystic fibrosis (CF) is the most common multisystem autosomal recessive genetic disorder with a high morbidity and mortality in Caucasian population with an incidence of approximately 1 of 3500 births worldwide [1]. Several studies were able to prove that quantification of liver stiffness by transient elastography (TE) is a novel, non-invasive method for the diagnosis of CFLD [3,4,5,6]. Longitudinal data of liver stiffness from patients with developing CFLD might allow to specify a cut-off for the rise in liver stiffness. We prospectively assessed in a 5-year follow-up whether transient elastography allows the identification of patients at risk for the progression of liver disease. For this purpose, patients received an annual quantification of liver stiffness by TE and the results were compared with two well-known fibrosis scores, APRI and FIB-4. Our results show that this approach has the potential to identify CF patients at risk of developing progression of CF liver disease
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