Magnetic resonance elastography to quantify liver disease severity in autosomal recessive polycystic kidney disease

Abdom Radiol (NY). 2021 Feb;46(2):570-580. doi: 10.1007/s00261-020-02694-1. Epub 2020 Aug 5.


Objectives: To evaluate whether liver and spleen magnetic resonance elastography (MRE) can measure the severity of congenital hepatic fibrosis (CHF) and portal hypertension (pHTN) in individuals with autosomal recessive polycystic kidney disease (ARPKD), and to examine correlations between liver MRE and ultrasound (US) elastography.

Methods: Cross-sectional study of nine individuals with ARPKD and 14 healthy controls. MRE was performed to measure mean liver and spleen stiffness (kPa); US elastography was performed to measure point shear wave speed (SWS) in both liver lobes. We compared: (1) MRE liver and spleen stiffness between controls vs. ARPKD; and (2) MRE liver stiffness between participants with ARPKD without vs. with pHTN, and examined correlations between MRE liver stiffness, spleen length, platelet counts, and US elastography SWS. Receiver operating characteristic (ROC) analysis was performed to examine diagnostic accuracy of liver MRE.

Results: Participants with ARPKD (median age 16.8 [IQR 13.3, 18.9] years) had higher median MRE liver stiffness than controls (median age 14.7 [IQR 9.7, 16.7 years) (2.55 vs. 1.92 kPa, p = 0.008), but MRE spleen stiffness did not differ. ARPKD participants with pHTN had higher median MRE liver stiffness than those without (3.60 kPa vs 2.49 kPa, p = 0.05). Liver MRE and US elastography measurements were strongly correlated. To distinguish ARPKD vs. control groups, liver MRE had 78% sensitivity and 93% specificity at a proposed cut-off of 2.48 kPa [ROC area 0.83 (95% CI 0.63-1.00)].

Conclusion: Liver MRE may be a useful quantitative method to measure the severity of CHF and pHTN in individuals with ARPKD.

Keywords: Autosomal recessive polycystic kidney disease; Congenital hepatic fibrosis; Magnetic resonance elastography; Portal hypertension; Ultrasound elastography.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Adolescent
  • Cross-Sectional Studies
  • Elasticity Imaging Techniques*
  • Humans
  • Liver / diagnostic imaging
  • Liver / pathology
  • Liver Cirrhosis / diagnostic imaging
  • Liver Cirrhosis / pathology
  • Magnetic Resonance Imaging
  • Polycystic Kidney, Autosomal Recessive* / diagnostic imaging
  • Severity of Illness Index