19-35: Intrahepatic Cholestasis of Pregnancy

Intrahepatic cholestasis of pregnancy is characterized by incomplete clearance of bile acids in genetically susceptible women. The principal symptom is pruritus, which can be generalized but tends to have a predilection for the palms and soles. Presentation is typically in the third trimester, and women with multi-fetal pregnancies are at increased risk. The finding of an elevated serum bile acid level, ideally performed in the fasting state, confirms the diagnosis. Associated laboratory derangements include modest elevations in hepatic transaminase levels and mild hyperbilirubinemia. Although rare, the bilirubin level may be sufficiently elevated to result in clinical jaundice. The symptoms and laboratory abnormalities resolve quickly after delivery but can recur in subsequent pregnancies or with exposure to combination oral contraceptives.

Adverse fetal outcomes, particularly preterm birth, nonreassuring fetal status, meconium-stained amniotic fluid, and stillbirth, have consistently been reported in women with cholestasis of pregnancy. The risk for adverse perinatal outcomes appears to correlate with disease severity as measured by the degree of bile acid elevation, and women with fasting bile acids greater than 40 mcmol/L have been reported to be at greatest risk. Because of the risks associated with cholestasis of pregnancy, many clinicians recommend antenatal testing in the third trimester and elective early delivery in attempt to avoid stillbirth. Evidence-based recommendations regarding such management practices, however, are not currently available. The use of ursodeoxycholic acid is not recommended for the treatment of intrahepatic cholestasis of pregnancy; a 2019 randomized controlled trial did not find that ursodeoxycholic acid reduced symptoms and morbidity for intrahepatic cholestasis of pregnancy.