Snapshot A 46-year-old obese woman presents to her gastrointestinal doctor for colicky right upper quadrant pain and pruritus. She denies having any weight loss, fevers, or chills. She does report having pruritus worse at night, as well as fatigue and light-colored stool. Labs show elevated alkaline phosphatase. A right upper quadrant ultrasound shows intrahepatic dilation of bile ducts and stones in the gallbladder. She is scheduled for a consultation with general surgery and started on ursodeoxycholic acid and cholestyramine. Introduction Overview secondary biliary cholangitis resulting from longstanding extrahepatic biliary obstruction Epidemiology Risk factors obstructive lesions gallstones biliary strictures malignancy pancreatic cancer inflammation pancreatitis ETIOLOGY Pathogenesis extrahepatic obstruction of the bile ducts causes ↑ pressure in the intrahepatic ducts this causes injury in the biliary tree, including fibrosis Presentation Symptoms common symptoms fatigue pruritus worse at night dark urine light-colored stool right upper quadrant pain Physical exam inspection jaundice hepatosplenomegaly may have xanthomas or xanthelasma Imaging Ultrasound indication to assess for hepatobiliary disease, including the etiology of extrahepatic obstruction views right upper quadrant findings gallstones intrahepatic vs extrahepatic ductal dilation Studies Serum labs liver function tests showing cholestasis pattern ↑ direct bilirubin ↑ alkaline phosphatase ↑ cholesterol Differential Primary biliary cholangitis key distinguishing factor + autoantibodies (anti-mitochondrial antibody) Hemochromatosis key distinguishing factor triad of cirrhosis, diabetes, and bronze skin pigmentation Wilson disease key distinguishing factors patients typically present before age 40 cirrhosis, neurologic disease, psychiatric disease, and Kayser-Fleischer rings in the eyes Treatment Management approach treat underlying condition i.e., cholecystectomy for cholelithiasis Medical ursodeoxycholic acid indications all patients cholestyramine indications severe pruritus refractory to other therapies Complications Ascending cholangitis