RESUMO
Liver disease in cystic fibrosis has many causes, with biliary fibrosis due to abnormal CFTR protein predominating. Assessment requires aetiology to be defined. Biliary fibrosis may progress to cirrhosis and portal hypertension, which although initially asymptomatic, may cause varices and splenomegaly. Monitoring progression includes clinical and ultrasound assessment with endoscopic assessment of varices for those at risk. Extrapolated primarily from longitudinal assessment of viral hepatitis in adults, non-invasive elastography has a potential role. Evidence is lacking to support intervention strategies, but ursodeoxycholic acid and ligation of varices are widely applied. Indication and timing of liver transplantation are not clearly defined. Multidisciplinary approach is needed to tailor assessment and guide management.