FRI088 Vanishing Bile Duct Syndrome?

Abstract

Abstract Disclosure: C.T. Malvar: None. D. Bleich: None. H. Moran: None. Background: Dyslipidemia is a common metabolic disorder in the US, but only a few conditions can cause a total cholesterol greater than 1000 mg/dl. We present a suspected case of vanishing bile duct syndrome in a patient referred to the emergency department due to transaminitis. Case Report: A 41-year-old female with type 2 diabetes mellitus and alcohol use disorder was found to have abnormal liver function tests. She had jaundice, pruritus, and bipedal edema for 2 weeks, but had quit drinking approximately 2 years ago. She denied any recent travel, sick contacts, prior history of hyperlipidemia, family history of premature CAD or lipid abnormalities. On chart review, she received Azithromycin 2 months before presentation for a presumed upper respiratory tract infection. On evaluation, she had scleral icterus and jaundice. Initial labs revealed Na 117 (133 - 145 meq/l), Cl 84 (97 - 110 meq/l), glucose of 542 (70 - 109 mg/dl), total bilirubin 20.9 (<=1.0 mg/dl), 218 (0 - 33 U/L), AST 133 (0 - 32 U/L), GGTP 778 (5 - 36 u/l), Alkaline phosphatase 2,327 (35 - 105 u/l), Lipase 252 (13 - 60 U/L), Total cholesterol 1329 (100 - 200 mg/dl), LDL 1282 (0 - 99 mg/dl), HDL 13, and Triglycerides 172 (0 - 200 mg/dL). Alpha 1 antitrypsin, Liver Kidney Microsomal (LKM-1) Antibody, ANA, Antimitochondrial antibody, Anti-smooth muscle antibody, Ceruloplasmin, CMV, EBV, HSV, and HIV were all within normal limits. An abdominal CT revealed a right pleural effusion and cholelithiasis without gallbladder wall thickening. A right upper quadrant ultrasound demonstrated hepatic steatosis. Magnetic resonance cholangiopancreatography (MRCP) showed no focal lesions, fatty infiltrations, or ductal dilatation. Ursodeoxycholic acid and cholestyramine were started for pruritus. Pseudohyponatremia was due to the increased lipid fraction in blood. Lipoprotein phenotyping revealed type IIb hypercholesterolemia with elevated triglycerides. An apo-lipoprotein B level was 147 mg/dl at the upper limit of normal (range 50-150 mg/dL). Cholestasis-induced hypercholesteremia was considered as a diagnosis. Endoscopy esophageal ultrasound with liver biopsy revealed a smooth liver with non-cirrhotic appearance, severe cholestatic pattern of injury, including bile duct infarcts, and marked bile ductular reaction. Ezetimibe was started instead of a statin due to elevated liver enzymes. Discussion: Vanishing bile duct syndrome is an extremely rare condition unknown to many clinicians. Patients present after drug-induced liver injury. Treatment focuses on symptomatic relief and supportive care. Hyperlipidemia in these patients minimally respond to statins and are thus avoided. The prognosis of these patients can vary from slow recovery to eventual cirrhosis with end-stage liver disease requiring transplant. Increasing awareness among physicians is essential to facilitate its diagnosis and management. Presentation: Friday, June 16, 2023