intrahepatic cholestasis of pregnancy (obstetric cholestasis)

Epidemiology

2nd most common cause of jaundice in pregnancy

Pathology

• multifactorial liver disorder of pregnancy
• cholestasis results from abnormal biliary transport from the liver into the small intestine

Genetics

• genetically determined
• associated with defects in ABCB4

Clinical manifestations

• generally presents in 2nd or 3rd trimester
• pruritus, often intense with or without jaundice
• jaundice may be profound
• pruritus may become more severe with advancing gestation & cholestasis
• fetal manifestations
  • fetal distress
  • spontaneous premature delivery
  • intrauterine death
• patients have spontaneous & progressive disappearance of cholestasis after delivery

Laboratory

• pattern of cholestasis
• elevated serum bile acids
  • indication for early delivery
• serum bilirubin elevated, < 6 mg/dL (case with serum bilirubin of 2.5 mg/dL)
• serum alkaline phosphatase markedly elevated
• serum AST < 200-500 U/L (case with serum ALT of 55 U/L)
• serum ALT < 200-500 U/L (case with serum AST of 30 U/L)
• complete blood count: no thrombocytopenia
• no evidence of hemolysis
• no evidence of disseminated intravascular coagulation

Complications

• benign disorder for mother, but associated with increased fetal mortality

Differential diagnosis

• fatty liver of pregnancy
• HELLP syndrome
• cholestatic features, pruritus & markedly increased serum alkaline phosphatase distinguishes

Management

• ursodeoxycholic acid
• cholestyramine for pruritus
• generally resolves promptly after delivery
• recurrence is common is subsequent pregnancies or with the use of oral contraceptives
• delivery at 1st sign of fetal distress

More general terms

• liver disease during pregnancy
• cholestasis
• dermatoses of pregnancy

Database

• OMIM: https://mirror.omim.org/entry/147480