International survey of expert practice patterns in the diagnosis and management of adult intestinal failure-associated liver disease.

BACKGROUND AND AIMS: Intestinal failure-associated liver disease (IFALD) remains difficult to define, diagnose, and manage in adults on home parenteral nutrition. We aimed to describe international expert practice patterns for IFALD monitoring, diagnosis, and treatment.

METHODS

Anonymous electronic survey was distributed to clinicians involved in HPN care. The survey collected respondent demographics, longitudinal monitoring practices after HPN initiation, and responses to adult clinical cases representing cholestatic, hepatocellular/steatotic, and advanced fibrotic clinical phenotypes. Because all clinical vignettes described adult patients, respondents were retained irrespective of their reported usual patient age group. Analyses were descriptive.

RESULTS

Seventy-two clinicians from 29 countries across 5 continents responded. Routine laboratory panels and liver function tests were obtained monthly or more often by 94.4% of respondents during the first 3 months of HPN, but practice shifted towards quarterly surveillance beyond 12 months. Total bilirubin, AST, ALT, alkaline phosphatase, and platelet count were nearly universal routine markers, whereas direct bilirubin and GGT were less consistently included. Initial cholestatic, persistent (6 months) cholestatic, hepatocellular/steatotic, and advanced fibrotic cases were diagnosed as IFALD by 43.1%, 84.7%, 37.1% and 77.9% of respondents, respectively. Ultrasound and elastography scan were the preferred adjunctive diagnostic assessments, yet many respondents reported uncertainty regarding elastography and imaging thresholds. Management centered on parenteral nutrition modification, especially changes in lipid emulsion, whereas transplant referral was uncommon except in advanced disease.

CONCLUSIONS

International expert practice is more consistent for surveillance intensity and lipid-focused management than for diagnostic thresholds and diagnostic work, particularly for non-cholestatic IFALD phenotypes. These findings support the development of phenotype-specific consensus, definitions, and validated adult diagnostic pathways for IFALD.