Prediction of chronic severe intestinal failure-associated liver disease by current criteria in adults: A descriptive cohort study.

Introduction: Intestinal failure-associated liver disease covers a spectrum of conditions from mild to end-stage disease. Currently, there are 9 diagnostic criteria divided to four categories: cholestasis, steatosis, fibrosis, and unclassified. Our aim was to evaluate the application of these criteria to patients with chronic severe liver disease in patients with intestinal failure.

Methods: This was a cross-sectional study of patients attending the home parenteral nutrition clinic of a national UK reference intestinal failure center from March 2015 to December 2019. Exclusion criteria included active malignancy, home parenteral nutrition for <6 months duration, and liver transplantation. Clinically significant intestinal failure-associated liver disease was defined as moderate-severe fibrosis or cirrhosis on liver biopsy and/or radiological imaging compatible with liver cirrhosis.

Results: Two hundred and twenty-one patients were included (age at home parenteral nutrition initiation: 50 ± 16.0 years; 63.6% female). There was a wide range of intestinal failure-associated liver disease point prevalence depending on the established criteria used (2.9%-35.1%). Twenty-three patients (9.5%) were diagnosed with clinically significant intestinal failure-associated liver disease, but no patient with clinically significant intestinal failure-associated liver disease met all diagnostic criteria, and 6 of 23 (26.1%) did not fit any of the established criteria.

Conclusions: Intestinal failure-associated liver disease is a poorly defined medical condition, and current noninvasive diagnostic methods are unreliable in predicting disease severity. Further studies are needed to develop the definition to reflect that intestinal failure-associated liver disease is a spectrum of disease that includes chronic severe liver disease and improve methods of disease diagnosis.