S5386 Elderly Onset Celiac Disease Presenting as Steatotic Liver Disease With Ascites

Introduction: Celiac disease (CD) is a multisystem disorder presenting with extraintestinal manifestations including hepatic abnormalities ranging from mild aminotransferase elevation to advanced fibrosis/cirrhosis. We present a case of elderly onset CD manifesting with steatotic liver disease (SLD), portal hypertension and ascites. Case Description/Methods: A 74-year-old woman with history of lymphocytic colitis presented with new ascites, weight loss, abdominal pain and diarrhea over 1 year. Liver chemistries showed alkaline phosphatase 106 U/L, alanine aminotransferase/aspartate aminotransferase 60/31 U/L, direct/total bilirubin 0.9/1.7 mg/dL, and serum albumin 2.1 g/dL. Liver duplex ultrasound showed severe steatosis and large ascites with patent hepatic vasculature. Paracentesis removed 5 L of ascitic fluid with serum-ascites albumin gradient 1.5 g/dL and total protein 1.5 g/dL. Body mass index was 19.3 with no previous history of obesity/metabolic syndrome or alcohol use. Serologic evaluation was notable for anti-smooth muscle antibody 1:1280, IgG 1632 mg/dL and tissue transglutaminase (TTG)-IgA 243 U/mL. Upper endoscopy showed villous atrophy and ulcerative enteritis, biopsies showed villous blunting, intraepithelial lymphocytosis, and patchy thickened subepithelial collagen table. Flow cytometry was negative for enteropathy-associated T-cell lymphoma. Transjugular liver biopsy with measured hepatic venous pressure gradient of 8 mmHg showed steatohepatitis with bridging fibrosis and no features of autoimmune hepatitis. The patient was supported with total parenteral nutrition, a strict gluten-free diet (GFD), and diuretics. After 6 months of a GFD she was off diuretics without recurrence of ascites, TTG-IgA improved to 43 U/mL, and ultrasound showed resolved hepatic steatosis. Discussion: Our case highlights a rare and severe hepatic manifestation of CD with improvement of portal hypertension after a GFD. There is an increased prevalence of SLD in CD patients adhering to a GFD despite lack of metabolic risk factors, however steatosis at diagnosis is noted in only 1.4% of patients. This may be aggravated by long standing intestinal protein malabsorption, also associated with SLD and described in patients with jejunoileal bypass. Elderly onset CD may have atypical patterns of clinical presentation; this case highlights the importance of considering CD in patients with liver disease, especially in older SLD patients without traditional risk factors.