212 Does our current diagnosis of cystic fibrosis related liver disease meet current diagnostic criteria?

Abstract

Background Although cystic fibrosis related liver disease (CFRLD) has emerged as the third most common cause of mortality in cystic fibrosis patients, it is currently poorly recognised. Aims To confirm the diagnosis of CFRLD in patients referred to the All Wales Adult Cystic Fibrosis Centre (AWACFC) and to ensure that no patients are being treated unnecessarily. Methods All patients holding a diagnosis of CFRLD attending the AWACFC were identified and their diagnosis of CFRLD was reviewed according to recognised diagnostic criteria (Debray et al 2011) alongside any prescription of ursodeoxycholic acid (UDCA) for them. Patients’ records were reviewed to ascertain whether patients had been investigated for other potential causes of liver disease. Results It was originally believed that 31% (78/249) of the CF patients at the AWACFC had a diagnosis of CFRLD. 45% (35/78) of these diagnoses were confirmed according to the recognised criteria. The true prevalence of CFRLD in our population is 14%. 55% of patients do not meet current criteria for CFRLD and 39% had been receiving UDCA unnecessarily. Conclusion Diagnoses of CFRLD should be regularly reviewed and confirmed using the approved diagnostic criteria and other causes of liver disease should be excluded. All deranged liver biochemistry may not be CFRLD and there is a degree of subjectivity to the interpretation of ultrasonography reports which could introduce a degree of inaccuracy with regard to the diagnosis of CFRLD. Despite recommendations stating that patients with suspected CFRLD should be investigated for other potential causes of liver disease less than a third of our cohort underwent these investigations.