NACFC 2021 | S03: Updates in Cystic Fibrosis Liver Disease

"Cystic fibrosis liver disease" is a widely used term that encompasses a broad range of liver abnormalities in CF, from hepatic steatosis to fibrosis/cirrhosis and cirrhotic/non-cirrhotic portal hypertension.

The methods used to diagnose "CFLD" have similarly been non-specific, leading to much confusion for providers, people with CF, and their families regarding what CFLD is and who has it. This session describes how imaging is transforming our ability to define and diagnose "CFLD" and provides tips on how to use different imaging modalities to diagnose different liver abnormalities in CF.

We describe the differences between "liver involvement," and "advanced liver disease," and discuss management strategies for advanced liver disease. This discussion highlights an increasingly appreciated entity – non-cirrhotic portal hypertension – and describes how it differs in cause and features from cirrhotic portal hypertension. Finally, there is much interest in how CFTR modulators may affect the liver and this symposium discusses existing and emerging data on this topic.

Originally recorded November 3, 2021.