101: Liver Disease in Pregnancy

Key Points

• Disease summary:

  • Hypertensive disorders of pregnancy

    • A spectrum of clinical disorders including pre-eclampsia, eclampsia, HELLP syndrome (hemolysis, elevated liver enzymes, low platelets), and pregnancy complicated by hepatic infarction and/or rupture.

    • Pre-eclampsia complicates approximately 5% of all pregnancies. Many women with pre-eclampsia have mildly abnormal liver function tests, but 5% to 20% of pre-eclamptic pregnancies develop HELLP syndrome.

    • Risk factors for the development of pre-eclampsia include primiparity, older maternal age, increased body mass index, long birth interval, medical disorders including diabetes mellitus, chronic renal failure and antiphospholipid syndromes, family history of pre-eclampsia, or fetal factors including multiple pregnancy, or the presence of a hydatidiform mole.

    • Uncomplicated pre-eclampsia is defined as the development of hypertension and proteinuria in pregnancy. Oedema is often seen. Pre-eclampsia can also be associated with abdominal pain, headaches, visual changes, and renal and liver impairment, but the liver impairment is usually mild.

    • HELLP syndrome is a subgroup of women with pre-eclampsia and is a severe variant of the condition. Hypertension and proteinuria may be absent in up to 15% of women presenting with features of HELLP syndrome. Severe disseminated intravascular coagulation and multiorgan failure can be associated with this condition and lead to significant maternal and fetal morbidity and mortality.

    • Hepatic hemorrhage or failure occurs in approximately 1% of pregnancies complicated by HELLP syndrome and are associated with significant mortality as fulminant liver failure may result.

    • All of these conditions are thought to have a common etiology, and result from abnormal placentation early in gestation, when the spiral arteries fail to form low-resistance vessels as they do in normal pregnancy. Diffuse endothelial activation occurs which can result in multiorgan dysfunction later in the course of the pregnancy.

    • Adverse fetal outcomes associated with hypertensive disorders in pregnancy include intrauterine growth restriction, placental abruption, and intrauterine death.

  • Acute fatty liver of pregnancy

    • This disorder is a rare and potentially life-threatening condition that occurs in pregnancy and usually presents toward the end of the third trimester.

    • Liver dysfunction results from microvesicular steatosis (in contrast to macrovesicular steatosis which is seen in other liver disorders such as nonalcoholic fatty liver disease).

    • More common in primigravidae, in multiple pregnancies, or pregnancies with male fetuses.

    • Important symptoms that suggest a diagnosis of acute fatty liver of pregnancy (AFLP) include abdominal pain (particularly in the right upper quadrant), nausea, vomiting, and polydipsia on a background of malaise and anorexia.

    • This disorder is considered part of the same spectrum as pre-eclampsia and HELLP syndrome and so the features may overlap. Severe hypertension or proteinuria is rare, however.

  • Intrahepatic cholestasis of pregnancy

    • Intrahepatic cholestasis of pregnancy (ICP) is the most frequent cause of cholestasis in pregnancy and is more commonly seen in certain populations (incidence in Chile and Scandinavia is 12% and 1.5%, respectively).

    • The disorder is characterized by the development of pruritus (in the absence of a rash) which can be severe, and deranged liver function tests, in association with elevated serum bile acid concentration.

    • It ...