Relevance of 3D Cholangiography and Transient Elastography to Assess Cystic Fibrosis-Associated Liver Disease?

Area of Science:

• Hepatology
• Radiology
• Gastroenterology

Background:

• Cystic fibrosis-associated liver disease (CFLD) is a significant cause of mortality in patients with cystic fibrosis.
• Early detection and accurate assessment of CFLD are crucial for patient management and improving outcomes.

Purpose of the Study:

• To evaluate the utility of magnetic resonance imaging (MRI) and liver stiffness measurement (LSM) in assessing CFLD.
• To determine the correlation between imaging findings, liver stiffness, and clinical parameters in CFLD patients.

Main Methods:

• Retrospective study including adult cystic fibrosis patients who underwent both MRI and LSM.
• Collected MR signs of portal hypertension (PHT), dysmorphia, and cholangitis.
• Liver stiffness was measured in kilopascals (kPa) and staged using the Metavir score.

Main Results:

• 52% of patients exhibited abnormal MRI findings.
• Median LSM was 5.7 kPa; 3 patients had F2 and 1 had F3 fibrosis scores.
• LSM was significantly higher in patients with PHT (7.85 kPa vs. 5 kPa, p=0.02).
• Abnormal liver function tests correlated with increased LSM (p=0.04).
• Seven patients had abnormal MRI findings despite normal ultrasonography results.

Conclusions:

• MRI and LSM are valuable tools for evaluating CFLD.
• These non-invasive methods can assist in screening for portal hypertension in CFLD patients.
• Combined MRI and LSM offer comprehensive insights into liver status in cystic fibrosis.