Review article: diagnosis and management of intestinal failure‐associated liver disease in adults

• Published in: Alimentary pharmacology & therapeutics 2019-09, Vol.50 (6), p.640-653
• Main Authors: Bond, Ashley, Huijbers, Angelique, Pironi, Loris, Schneider, Stephane M., Wanten, Geert, Lal, Simon
• Format: Article
• Language: English
• Subjects: Adult; Animals; Biopsy; Cholestasis; Cirrhosis; Diagnosis; Fatty liver; Fibrosis; Grafts; Humans; Intestinal Diseases - complications; Intestinal Diseases - diagnosis; Intestinal Diseases - therapy; Intestine; Liver; Liver cirrhosis; Liver diseases; Liver Diseases - diagnosis; Liver Diseases - etiology; Liver Diseases - therapy; Liver transplantation; Management; Morbidity; Parenteral nutrition; Steatosis; Transplantation
• ISSN: 0269-2813; 1365-2036
• DOI: 10.1111/apt.15432

Summary Background Hepatic disturbances in the context of intestinal failure and parenteral nutrition (PN) are frequently encountered and carry a significant burden of morbidity and sometimes mortality. The term intestinal failure‐associated liver disease (IFALD) refers to liver injury due to intestinal failure and associated PN, in the absence of another evident cause of liver disease, encompassing a spectrum of conditions from deranged liver enzymes, steatosis/ steatohepatitis, cholestasis as well as progressive fibrosis, cirrhosis and end‐stage liver disease. Aims To present an up to date perspective on the diagnosis/definition, aetiologies and subsequent management of IFALD and to explore future consideration for the condition, including pharmacological therapies Results In adults using long‐term PN for benign chronic intestinal failure, 1%‐4% of all deaths are attributed to IFALD. The aetiology of IFALD is multifactorial and can be broadly divided into nutritional factors (eg lipid emulsion type) and patient‐related factors (eg remaining bowel anatomy). Given its multifaceted aetiology, the management of IFALD requires clinicians to investigate a number of factors simultaneously. Patients with progressive liver disease should be considered for combined liver‐intestine transplantation, although multivisceral grafts have a worse prognosis. However, there is no established non‐invasive method to identify progressive IFALD such that liver biopsy, where appropriate, remains the gold standard. Conclusion A widely accepted definition of IFALD would aid in diagnosis, monitoring and subsequent management. Management can be complex with a number of factors to consider. In the future, dedicated pharmacological interventions may become more prominent in the management of IFALD.