A 44-year-old woman comes to the office due to fatigue. Medical history is notable for hypertension, obesity, and hypothyroidism; current medications include hydrochlorothiazide, amlodipine, and levothyroxine. The patient does not use tobacco but drinks 1 or 2 glasses of wine on weekends. She stopped using recreational drugs approximately 10 years ago.
Examination shows multiple skin xanthelasmas, a few scattered hyperpigmented spots, and tenderness in the right upper quadrant of the abdomen. The liver is palpable 3 cm below the costal margin and mild splenomegaly is present.
Laboratory results are as follows:
Which of the following is this patient at the highest risk of developing?

Question

A 44-year-old woman comes to the office due to fatigue.  Medical history is notable for hypertension, obesity, and hypothyroidism; current medications include hydrochlorothiazide, amlodipine, and levothyroxine.  The patient does not use tobacco but drinks 1 or 2 glasses of wine on weekends.  She stopped using recreational drugs approximately 10 years ago.
Examination shows multiple skin xanthelasmas, a few scattered hyperpigmented spots, and tenderness in the right upper quadrant of the abdomen.  The liver is palpable 3 cm below the costal margin and mild splenomegaly is present.
Laboratory results are as follows:
 Which of the following is this patient at the highest risk of developing?

Quizplus · Accepted Answer

11ec193a_97f6_6f24_853d_af2c1438be4d_MD0002_00 This patient's presentation is consistent with primary biliary cholangitis (PBC, formerly primary biliary cirrhosis). PBC is due to autoimmune destruction of intralobular bile ducts and predominantly affects women age 30-65. Patients with PBC typically have a cholestatic injury pattern on liver biomarkers (disproportionate elevation in alkaline phosphatase and bilirubin compared to transaminases), and anti-mitochondrial antibodies are positive in ~95% of cases. Antinuclear antibodies are present in approximately 70% of patients and correlate with a poor prognosis. The diagnosis can be confirmed with liver biopsy. Up to 50% of patients develop metabolic bone disease, which may manifest as osteoporosis and/or osteomalacia. The cause of osteoporosis in PBC is unknown but is thought to be due to cholestasis leading to an accumulation of substances that inhibit bony turnover. Additionally, the disease predominately affects middle aged female patients who are already at risk for osteoporosis. Patients may have malabsorption of fat-soluble vitamins, but those with PBC-related osteoporosis usually have normal vitamin D levels. Nevertheless, patients with PBC should receive routine supplementation with calcium and vitamin D in addition to regular bone density screening. (Choices A and D) This patient has evidence of prior hepatitis B infection and recovery, with positive hepatitis B surface and core antibodies. Aplastic anemia may rarely occur in hepatitis B, but it is not usually a complication of PBC (this patient's mild thrombocytopenia is most likely due to portal hypertension). Polyarteritis nodosa is a possible complication of chronic hepatitis B but not PBC. (Choice B) Cryoglobulinemia is typically associated with hepatitis C, but it is not usually seen with PBC. This patient has a negative hepatitis C antibody screen. (Choice E) Patients with PBC can develop significant hypercholesterolemia (sometimes >1,000 mg/dL), which leads to xanthelasmata and xanthomas. However, these patients are not at increased risk for coronary artery disease, possibly due to marked elevation of HDL but only mildly elevated LDL and VLDL. Educational objective: Primary biliary cholangitis predominantly affects women age 30-65. Patients typically have a cholestatic injury pattern on liver biomarkers and positive anti-mitochondrial antibodies. Up to 50% of patients also develop metabolic bone disease, which may manifest as osteoporosis and/or osteomalacia. __________ References: Osteoporosis in patients with primary biliary cirrhosis. (http://www.ncbi.nlm.nih.gov/pubmed/20926953) Primary biliary cirrhosis. (http://www.ncbi.nlm.nih.gov/pubmed/21601785)

A 44-Year-Old Woman Comes to the Office Due to Fatigue