A 22-year-old woman is evaluated for intractable pruritus that keeps her awake at night. She is in her 25th week of pregnancy. Her medications are a prenatal vitamin and a folate supplement.
On physical examination, vital signs are normal. Scleral icterus is noted. Excoriations are seen on the arms, chest, abdomen, and legs.
|Platelet count||370,000/μL (370 × 109/L)|
|Alanine aminotransferase||55 U/L|
|Aspartate aminotransferase||30 U/L|
|Total bilirubin||2.5 mg/dL (42.8 µmol/L)|
Testing for hepatitis C and hepatis B viral infections is negative.
An ultrasound shows a normal liver with no dilated bile ducts.
Which of the following is the most appropriate next step in management?
A. Liver biopsy
B. Peripheral blood smear
C. Topical glucocorticoids
D. Ursodeoxycholic acid
MKSAP Answer and Critique
The correct answer is D. Ursodeoxycholic acid. This content is available to MKSAP 18 subscribers as Question 60 in the Gastroenterology and Hepatology section. More information about MKSAP is available online.
Ursodeoxycholic acid therapy is the most appropriate management for this patient. Intrahepatic cholestasis of pregnancy is a liver condition that affects pregnant women in the second or third trimester. This condition spontaneously improves within 48 hours of delivery. Typical symptoms include diffuse pruritus, and there is typically a mild increase in serum transaminase concentrations. Associated risks include premature delivery as well as higher rates of fetal death. Mutations in bile salt transporters have been implicated in the pathophysiology. The mainstay of therapy for intrahepatic cholestasis of pregnancy is ursodeoxycholic acid, which is associated with alleviated symptoms, improved liver test abnormalities but did not change fetal outcomes.
Cholestasis of pregnancy can be diagnosed in pregnant women with otherwise unexplained pruritus and abnormal liver chemistry tests. Elevated levels of total serum bile acid in a pregnant woman with pruritus are considered diagnostic for cholestasis of pregnancy in the absence of an alternative diagnosis. In this patient with findings compatible with intrahepatic cholestasis of pregnancy and a normal hepatic ultrasound, a liver biopsy is not needed.
HELLP (Hemolysis, Elevated Liver enzymes, and Low Platelets) syndrome presents during the third trimester and is an advanced complication of preeclampsia. HELLP syndrome typically presents with abdominal pain, new-onset nausea and vomiting, pruritus, and jaundice. A peripheral blood smear will reveal schistocytes. This patient is not anemic and does not have thrombocytopenia or hypertension; therefore, a peripheral blood smear to support the diagnosis of HELLP syndrome is not needed.
Pruritic urticarial papules and plaques of pregnancy (PUPPP) is the most common specific dermatosis of pregnancy. PUPPP is a clinical diagnosis confirmed by the appearance late in the third trimester of erythematous plaques in the distribution of striae. Persistent and bothersome pruritus is a symptomatic hallmark of the condition. It is not associated with jaundice or elevated transaminase levels. The condition usually resolves shortly after delivery. The first-line option for therapy is usually topical glucocorticoids of low to mid potency. Topical glucocorticoids are of no value in intrahepatic cholestasis of pregnancy.
- The mainstay of therapy for intrahepatic cholestasis of pregnancy is ursodeoxycholic acid, which is associated with alleviated symptoms and improved liver test abnormalities.