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.
Ursodeoxycholic acid (8–10 mg/kg/day) is the treatment of choice and results in decreased pruritus in most women. Although its mechanism of action is not entirely understood, this medication has also been demonstrated to decrease serum bile acids in treated women and does not appear to have any adverse effects on the fetus. Other medications have been examined in small studies, but none are as effective in improving symptoms and reducing biomarkers and are not, therefore, considered first-line therapy.
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.
et al. Intrahepatic cholestasis of pregnancy: review of six national and regional guidelines. Eur J Obstet Gynecol Reprod Biol. 2018 Dec;231:180–7.
et al. Intrahepatic cholestasis of pregnancy: a review of diagnosis and management. Obstet Gynecol Surv. 2018 Feb;73(2):103–9.