A 45-year-old woman is noted to have abnormal liver chemistries. She has no symptoms. The patient has hypercholesterolemia and her only medication is a statin. She lives with her husband and 3 children and works in a grocery store. Temperature is 37 C (98.6 F), blood pressure is 118/72 mm Hg, pulse is 68/min, and respirations are 12/min. BMI is 25 kg/m². Physical examination reveals no abnormalities. Laboratory results are as follows: Right upper quadrant ultrasonogram shows no abnormalities. After confirmation of the diagnosis, which of the following is the best next step in management of this patient?

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11ec25c8_d402_e86c_bc81_939e0dab14fa_MD0003_00 This asymptomatic patient has elevated alkaline phosphatase with normal hepatic transaminase (alanine aminotransferase [ALT], aspartate aminotransferase [AST]) levels, normal right upper quadrant ultrasound, and positive antimitochondrial antibody assay. This is consistent with early primary biliary cholangitis (PBC, previously primary biliary cirrhosis), a chronic, progressive liver disease characterized by cholestasis with autoimmune destruction of intrahepatic bile ducts. It is most common in middle-aged women and has an insidious onset. Pruritus and fatigue are usually the first symptoms. As the disease progresses, jaundice, steatorrhea, hepatomegaly, eyelid xanthelasma, portal hypertension, and osteopenia may develop. Ursodeoxycholic acid (UDCA) is used in a number of cholestatic disorders and is the drug of choice in PBC. UDCA is a hydrophilic bile acid that decreases biliary injury by the more hydrophobic endogenous bile acids. It also increases biliary secretion and may have additional anti-inflammatory and immunomodulatory effects. UDCA delays histologic progression in PBC and may improve symptoms and possibly survival. It should be initiated as soon as the diagnosis is made, even in asymptomatic patients. Treatment is less effective in advanced disease, and many patients will go on to require liver transplantation. (Choice A) Statins cause a hepatocellular rather than cholestatic pattern of injury, characterized by elevated liver transaminases (ALT, AST). This patient has normal transaminases and an elevated alkaline phosphatase level. (Choice B) Alpha-fetoprotein (AFP) is used to screen for hepatocellular carcinoma in patients with chronic viral hepatitis or cirrhosis. (Choice C) Liver transplantation is the definitive cure for progressive PBC but is indicated only in those with severe liver damage or cirrhosis. As this patient has no signs or symptoms of cirrhosis, a trial of UDCA is more appropriate. (Choice D) Glucocorticoids are used for autoimmune hepatitis, which is characterized by elevated liver transaminases and a positive antinuclear antibody titer. Glucocorticoids are ineffective in PBC. Educational objective: Ursodeoxycholic acid (UDCA) is the drug of choice in primary biliary cholangitis (PBC). It delays histologic progression and may improve symptoms and possibly survival. Therefore, UDCA should be initiated as soon as the diagnosis of PBC is made. Patients with advanced disease require liver transplantation. References: Primary biliary cirrhosis. (http://www.ncbi.nlm.nih.gov/pubmed/26364546)

A 45-Year-Old Woman Is Noted to Have Abnormal Liver Chemistries