Non-Invasive Evaluation of Cystic Fibrosis Related Liver Disease in Adults with ARFI, Transient Elastography and Different Fibrosis Scores

Abstract

BackgroundCystic fibrosis-related liver disease (CFLD) is present in up to 30% of cystic fibrosis patients and can result in progressive liver failure. Diagnosis of CFLD is challenging. Non-invasive methods for staging of liver fibrosis display an interesting diagnostic approach for CFLD detection.AimWe evaluated transient elastography (TE), acoustic radiation force impulse imaging (ARFI), and fibrosis indices for CFLD detection.MethodsTE and ARFI were performed in 55 adult CF patients. In addition, AST/Platelets-Ratio-Index (APRI), and Forns' score were calculated. Healthy probands and patients with alcoholic liver cirrhosis served as controls.ResultsFourteen CF patients met CFLD criteria, six had liver cirrhosis. Elastography acquisition was successful in >89% of cases. Non-cirrhotic CFLD individuals showed elastography values similar to CF patients without liver involvement. Cases with liver cirrhosis differed significantly from other CFLD patients (ARFI: 1.49 vs. 1.13 m/s; p = 0.031; TE: 7.95 vs. 4.16 kPa; p = 0.020) and had significantly lower results than individuals with alcoholic liver cirrhosis (ARFI: 1.49 vs. 2.99 m/s; p = 0.002). APRI showed the best diagnostic performance for CFLD detection (AUROC 0.815; sensitivity 85.7%, specificity 70.7%).ConclusionsARFI, TE, and laboratory based fibrosis indices correlate with each other and reliably detect CFLD related liver cirrhosis in adult CF patients. CF specific cut-off values for cirrhosis in adults are lower than in alcoholic cirrhosis.

Serum Proteome Profiling Identifies Novel and Powerful Markers of Cystic Fibrosis Liver Disease

“…Interestingly, with a mean liver stiffness of 9.95 kPa in patients with CFLD compared to 4.3 kPa in those without CFLD, we found very similar data in our total patient cohort compared to those described by Witters and colleagues. Furthermore, a cut-off of 6.3 kDa was calculated to be optimal for the detection of CFLD within the present study, thereby corroborating the cut-off defined in previous studies on the detection of CFLD by TE [14], [21].…”

“…As a further means for the assessment of the expression of serum fibrosis markers in CFLD, we measured liver stiffness values by transient elastography (TE) in our patient cohort, which has been proven to accurately diagnose liver disease and hepatic fibrosis of various etiologies, including CFLD [13], [14], [20], [21]. For this purpose we initially assessed the diagnostic value of TE in our CF patient cohort.…”

“…Specifically, in CFLD, Witters and co-workers recently demonstrated that adults and children with CFLD had a significantly elevated mean liver stiffness of 8.8 kPa compared to 5 kPa in individuals without CFLD [13]. In a very recent report, it was further shown that CF patients with C FL D had increased TE values compared to patients without CFLD, with highest liver stiffness measurements seen in patients with cirrhosis [21]. Interestingly, with a mean liver stiffness of 9.95 kPa in patients with CFLD compared to 4.3 kPa in those without CFLD, we found very similar data in our total patient cohort compared to those described by Witters and colleagues.…”

Impact of Liver Disease After the Fontan Operation

“…INR values were not recorded as the majority of patients were on warfarin therapy. Several scores found to be predictive of hepatic fibrosis in other patient populations (7)(8)(9) were calculated including AST to ALT ratio (7), APRI (AST to platelet ratio) (8) and FIB-4 score (age*AST/platelet count*ALT) (9).…”

Asian-Pacific Association for the Study of the Liver (APASL) consensus guidelines on invasive and non-invasive assessment of hepatic fibrosis: a 2016 update

“…However, detection of CF-associated liver disease (CFLD) is challenging. Many studies have been done in the recent years to assess the role of different non-invasive markers in assessing liver fibrosis and PHT in patients with CF [177][178][179][180][181][182] [181]. Furthermore, TIMP-1 had a high diagnostic accuracy for CFLD in adults.…”