S5675 Rapidly Progressive Vanishing Bile Duct Syndrome in an Elderly Woman Without Identifiable Risk Factors: A Case Report

Introduction: Vanishing bile duct syndrome (VBDS) is an uncommon, acquired cholestatic hepatic disease characterized by the destruction and loss of intrahepatic bile ducts in >50% of the portal areas. Several inciting factors for it have been identified, and the prognosis depends largely on the underlying etiology and the extent of bile duct injury. In this report, we present a challenging case of idiopathic VBDS with rapid progression to liver failure. Case Description/Methods: A 65-year-old woman patient with a history of diabetes mellitus presented to our emergency department complaining of jaundice and vague right upper quadrant abdominal pain. She denied any history of alcohol or drug intake apart from insulin prior to admission. An abdominal ultrasound excluded the presence of stones, and a follow-up MRCP confirmed an average caliber of the biliary tree. Aside from scratch marks because of cholestatic pruritis, her physical examination was otherwise insignificant. Upon testing, she had shooting cholestatic markers with moderate transaminitis, however, her virology panel, autoimmune work-up and tumor markers were negative. Subsequently, a liver biopsy was performed that confirmed the presence of ductopenia and therefore, a diagnosis of VBDS was made. The patient was started on ursodeoxycholic acid with initial significant decline of bilirubin from 38.7 mg/dl to 17.7 mg/dl before rising slightly again to reach a plateau of 20.4 mg/dl. She stopped responding to treatment after 3 weeks with rapid deterioration over the course of 2 months. She rapidly progressed to liver failure with elevated prothrombin time and international normalized ratio, and exhibited signs of hepatic encephalopathy prior to her demise while awaiting liver transplantation. Discussion: VBDS is a rare, acquired clinical disease with a poorly understood pathophysiology. In addition to being drug-induced, it has been linked to underlying autoimmune diseases, GVHD, malignancies, or in exceedingly rare cases, it can be idiopathic. In most cases, VBDS tends to be slowly progressive over months with the treatment focused on controlling the triggering condition. Our report is a peculiar deviation from established literature in that it represents a rapidly progressive case with an acute decompensation without any identifiable risk factors. It is imperative to include VBDS in the early, broad differential of cholestasis as timely diagnosis and intervention can be crucial for a more favorable prognosis.