A 58-year-old woman comes to the office due to progressive fatigue over the last 2 years. She has also experienced generalized pruritus, for which she has used different lotions over the past year, but she has had no chest pain, abdominal pain, diarrhea, or weight loss. The patient takes lansoprazole for acid reflux, atorvastatin for hypercholesterolemia, and fosinopril for hypertension. She received a blood transfusion following a motor vehicle collision several years ago. The patient has no history of illicit drug use. She was adopted and her biological history is unknown.
Temperature is 36.7 C (98 F) , blood pressure is 144/89 mm Hg, and pulse is 78/min. BMI is 27 kg/m². The patient appears healthy and well tanned although she denies sun exposure. There is no scleral icterus. Cardiac and pulmonary examinations are within normal limits. There is mild hepatomegaly.
Laboratory results are as follows:
Which of the following is most likely in this patient?

Question

A 58-year-old woman comes to the office due to progressive fatigue over the last 2 years. She has also experienced generalized pruritus, for which she has used different lotions over the past year, but she has had no chest pain, abdominal pain, diarrhea, or weight loss. The patient takes lansoprazole for acid reflux, atorvastatin for hypercholesterolemia, and fosinopril for hypertension. She received a blood transfusion following a motor vehicle collision several years ago. The patient has no history of illicit drug use. She was adopted and her biological history is unknown.
Temperature is 36.7 C (98 F) , blood pressure is 144/89 mm Hg, and pulse is 78/min. BMI is 27 kg/m². The patient appears healthy and well tanned although she denies sun exposure. There is no scleral icterus. Cardiac and pulmonary examinations are within normal limits. There is mild hepatomegaly.
Laboratory results are as follows:

Which of the following is most likely in this patient?

Quizplus · Accepted Answer

11ec193a_9803_8da8_853d_594dd39fec6b_MD0002_00 This patient has fatigue, pruritus, and a cholestatic pattern on liver function tests (elevation of alkaline phosphatase and gamma-glutamyl transferase disproportionate to elevations in transaminases). In a young or middle-aged woman, this is consistent with primary biliary cholangitis (PBC; previously known as primary biliary cirrhosis). PBC is a progressive autoimmune disease that leads to fibrosis and destruction of the intrahepatic bile ducts. It is most common in women age 30-65. Over 50% of patients are asymptomatic at diagnosis and detected from typical biochemical abnormalities on liver function tests done for other purposes. Among symptomatic patients, fatigue and generalized pruritus (which may be worse at night) predominate. Marked hyperlipidemia may also be present, leading to formation of xanthomas and xanthelasmas. Also, patients often have additional autoimmune disorders (eg, CREST syndrome, type 1 diabetes mellitus, rheumatoid arthritis). Antimitochondrial antibodies (AMA) are highly sensitive and specific for PBC, and are diagnostic in the setting of cholestasis. Liver biopsy is indicated if AMA are negative or for staging purposes, but is not required for diagnosis in patients with biochemical and clinical evidence of PBC. (Choices B, C, D, and E) Patients with PBC often have hyperpigmentation due to abnormal melanin deposition. This may lead to mistaken suspicion for hereditary hemochromatosis. However, hemochromatosis is characterized by a hepatocellular injury pattern (predominant elevations in transaminases) rather than a cholestatic pattern. Other disorders characterized by a hepatocellular rather than cholestatic pattern include alcoholic liver disease, viral hepatitis (eg, hepatitis C), and statin-induced liver injury. Educational objective: Primary biliary cholangitis (PBC) is a progressive autoimmune disease leading to fibrosis and destruction of the intrahepatic bile ducts, and is usually seen in women age 30-65. Clinical features may include pruritus, fatigue, skin hyperpigmentation, hyperlipidemia, and elevated alkaline phosphatase levels. Antimitochondrial antibodies have high sensitivity and specificity for PBC. __________ References: Diagnosis of primary biliary cirrhosis. (http://www.ncbi.nlm.nih.gov/pubmed/22117636)

A 58-Year-Old Woman Comes to the Office Due to Progressive