Abstract
ObjectivesStaging of fibrosis in chronic liver disease is important for prognosis and treatment planning. Liver biopsy is the gold standard in fibrosis assessment; however, new methods for fibrosis and stiffness measurement exist which have not been evaluated in patients with Wilson’s disease. To evaluate the accuracy of collagen proportionate area (CPA), transient elastography and shear wave elastography (SWE) in the assessment of liver fibrosis in adult patients with Wilson’s disease.MethodsIn this retrospective study of 60 patients with Wilson’s disease, results of percutaneous cutting liver biopsy assessed using the Ishak fibrosis score and CPA were compared with liver stiffness measured with transient elastography and SWE.ResultsCPA correlated with the Ishak score (r = 0.45; P = 0.001) and transient elastography results correlated with SWE measurements (r = 0.80; P = 0.0001). In contrast, transient elastography or SWE did not significantly correlate with the Ishak score or CPA.ConclusionCollagen content assessment may be useful for estimation of liver fibrosis in patients with Wilson’s disease. However, single time-point elastographic liver stiffness measurements have a limited diagnostic value in Wilson’s disease.
Highlights
Wilson’s disease is an autosomal recessive inherited disease of impaired excretion and excessive accumulation of copper caused by mutations in the ATP7B gene that encodes the ATP7B copper transporter [1]
The study included patients who had percutaneous liver biopsy, transient elastography and shear wave elastography (SWE) to assess liver fibrosis within a period of six months and who had not been diagnosed with liver cirrhosis previously
There was no significant correlation between measurements of collagen proportionate area (CPA), transient elastography or SWE and any of the clinical features and laboratory prognostic scores tested (De Ritis ratio, FIB-4, AST-to-platelet ratio index (APRI), Bonacini and model of end-stage liver disease (MELD)) (Table 3)
Summary
Wilson’s disease is an autosomal recessive inherited disease of impaired excretion and excessive accumulation of copper caused by mutations in the ATP7B gene that encodes the ATP7B copper transporter [1]. The worldwide incidence of Wilson’s disease is estimated at 1:30 000 [2]. Accumulation of copper mostly affects the liver, brain and cornea, leading to organ injury. The clinical presentation of liver disease in Wilson’s disease spans asymptomatic elevation of liver enzymes, acute or chronic hepatitis, occult cirrhosis and fulminant hepatic failure [3,4]. Assessment of liver fibrosis in chronic liver diseases has an essential prognostic value. Liver biopsy is the gold standard for diagnosis of liver cirrhosis and fibrosis and several histopathologic scoring systems exist that are European Journal of Gastroenterology & Hepatology 2021, 33:535–540
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