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Old 05-26-2013
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Liver Gastroenterology Q20

48-year-old woman presents complaining of fatigue and itching. She has been tired for the past 6 months and has recently developed diffuse itching. It is worse in the evening hours, but it is intermittent. She does not note it to be worse following hot baths or showers. Her past medical history is significant only for hypothyroidism for which she takes levothyroxine 125 μg daily. On physical examination, she has mild jaundice and scleral icterus. The liver is enlarged to 15 cm on palpation and is palpable 5 cm below the right costal margin. Xanthomas are seen on both elbows. Hyperpigmentation is noticeable on the trunk and arms where the patient has excoriations. Laboratory studies demonstrate the following: WBC 8900/μL, hemoglobin 13.3 g/dL, hematocrit 41.6%, and platelets 160,000/μL. The creatinine is 1.2 mg/dL. The AST is 52 U/L, ALT is 62 U/L, alkaline phosphatase is 216 U/L, total bilirubin is 3.2 mg/dL, and direct bilirubin is 2.9 mg/dL. The total protein is 8.2 g/dL, and albumin is 3.9 U/L. The thyroid-stimulating hormone is 4.5 U/mL.
Antimitochondrial antibodies are positive. P-ANCA and C-ANCA are negative. What is the most likely cause of the patient’s symptoms?
A. Lymphoma
B. Polycythemia vera
C. Primary biliary cirrhosis
D. Primary sclerosis cholangitis
E. Uncontrolled hypothyroidism
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Old 05-26-2013
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This is a common presentation for PBC.. extremely common in women.. pruritus appears before jaundice and it can also explain the excoriations she has (excessive scratching)
Treatment is with ursodeoxycholic acid and liver transplantation
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Old 05-27-2013
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answer is C.

Primary biliary cirrhosis

The clinical presentation is consistent with a cholestatic picture, which can present with painless jaundice and pruritus. The pruritus can be prominent and is present in 50% of individuals at the time of diagnosis. The pruritus is typically intermittent and worse in the evening. There is no other prominent associa-tion such as following hot baths or showers, which occurs in polycythemia vera. Other causes of pruritus outside of cholestasis include lymphoma and uncontrolled hypo-or hyperthyroidism. However, the laboratory studies in this patient clearly represent cholestasis with an elevation in alkaline phosphatase and bilirubin. The clinical char-acteristics are more commonly seen in primary biliary cirrhosis compared to primary sclerosis cholangitis, as the patient is a middle-aged female with positive antimito-chondrial antibodies. In contrast, primary sclerosing cholangitis is associated with positive perinuclear antineutrophil cytoplasmic antibodies in 65% of patients, and 50% of individuals with primary sclerosing cholangitis have a history of ulcerative colitis.
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