S3050 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

Abstract

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, NH3 182 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. (Figure) 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.Figure 1.: Liver biopsy showing cirrhosis with marked steatohepatitis, mild lobular inflammation, ballooning hepatocyte degeneration nodular and peri-cellular fibrosis stage IV Table 1. - Differentiating features between IFALD vs NAFLD FEATURES IFALD NAFLD Metabolic syndrome No metabolic syndrome ,no insulin resistance, or it is improved after initiating PN, low BMI and low plasma cholesterol levels Metabolic syndrome common with Insulin resistance, hyperlipidemia high BMI Nutritional status and PN dependence Severe malabsorption, mostly dependent on PN No malabsorption, PN mostly not required Plasma choline levels Low plasma free choline concentration Normal to high levels Effect of choline supplementation Reduction of steatosis ,improved liver tests with choline supplementation Minimal difference to choline supplementation Cholestasis Highly evident with hyperbilirubinemia Not typical Steatosis (macro vs micro) Macro and micro steatosis Predominantly macro steatosis Disease progression and cirrhosis development Rapid progression to ESLD, cirrhosis develops within ∼3-5 months after initiating PN Longer duration ∼10-20 years for cirrhosis to develop Zone of steatosis More common in zone -1 (periportal) Mostly involves zone -1(peri-central) Pattern of fibrosis Characteristic "jig-saw" pattern fibrosis Sinusoidal fibrosis with ballooning of hepatocytes Treatment Intestinal transplant is mainstay of treatment to overcome malabsorption and impending liver failure No role of intestinal transplant as no malabsorption. Prognosis Rapid onset of death within 1-4 years Rapid death extremely rare