C0574 - Intestinal Failure-Associated Liver Disease vs Non Alcoholic Fatty Liver Disease in the Setting of Short Bowel Syndrome: A Case of Rapidly Progressive Hepatic Failure
University of Illinois at Chicago Chicago, Illinois
Introduction: Intestinal Failure-Associated Liver Disease (IFALD) is a progressive disease with a high mortality rate in patients dependent on parenteral nutrition (PN). It is a multifactorial entity associated with a spectrum of hepatic manifestations including cholestasis, steatosis, portal hypertension, choline deficiency and manganese toxicity. In patients with Short Bowel Syndrome (SBS), hepatic steatosis occurs in 40-55% and IFALD in 5-15%. Risk factors for IFALD in SBS include chronic PN use and length of remaining bowel.Early IFALD may present similarly to NAFLD, however pathogenic and prognostic differences make distinguishing these diseases crucial. We present a case of rapidly progressive IFALD in an adult SBS patient after PN use.
Case Description/Methods: We present a 65 year-old female with massive short bowel resection (< 30cm remaining) and malnutrition with a 12-year history of on-and-off PN use. PN was discontinued 6 months prior and she was gaining weight with enteral feeding and teduglutide. She presented with right upper quadrant pain and nausea. Ultrasound showed cholelithiasis and hepatic steatosis. She was discharged after resolution of symptoms. In two weeks, she returned with jaundice, worsening abdominal pain, weight loss, altered mentation and asterixis. Labs showed total bilirubin 11.4 mg/d L (Direct 6.0mg/d L), ALP 139 IU/L, AST 136 IU/L, ALT 65 IU/L, NH3182 mg/L and serum carnitine 15 μmol/L. She was treated for hepatic encephalopathy with lactulose, rifaximin and carnitine. Computed tomography showed moderate ascites, mesenteric edema, and edematous bowels. Diagnostic paracentesis revealed portal hypertension. Liver biopsy showed cirrhosis with steatohepatitis and peri-cellular fibrosis consistent with TPN-associated liver disease in the setting of SBS-IF(Image A). Multifocal pneumonia with multi-organ failure led to her death.
Discussion: This case demonstrates the potentially rapid progression of IFALD, particularly in patients with SBS. Clinicians should exercise high clinical suspicion of IFALD in patients with a history of PN use and SBS that present with hepatic manifestations. Early recognition is important to distinguish the disease from similarly presenting NAFLD (table 1). It is also vital to consider and treat other factors that may exacerbate hepatic disease including nutritional deficiencies. There may be benefit to diagnosis with liver biopsy early in the disease course to initiate prompt treatment or transplant, preventing rapid and fatal progression.
