Differential diagnosis for acute liver disease in pregnancy – a suspected case of acute fatty liver of pregnancy

Introduction Acute fatty liver of pregnancy (AFLP) is rare and potentially fatal for mother and child. It typically occurs in the third trimester of pregnancy. The pathophysiology is incompletely understood and causal therapy is termination of the pregnancy.

Differential diagnosis for acute liver disease in pregnancy includes unrelated liver disease that coincides with pregnancy, intrahepatic cholestasis of pregnancy, preeclampsia and syndrome of hemolysis, elevated liver enzymes, and low platelet count (HELLP).

Case Description A 35-year-old primigravida with no prior history of liver disease presented to our clinic in 38+4 weeks of gestation with jaundice, upper abdominal pain, nausea and vomiting since about one week after an uneventful pregnancy. She denied headaches, visual disturbances or itching. Blood pressure was normal at all times. Fetal Ultrasound and CTG were normal. An ultrasound of the maternal liver was normal. At admission, laboratory findings showed beginning liver failure with elevated aminotransferases and coagulopathy, hyperbilirubinemia, acute kidney injury and hemolysis. sFlt-1/PlGF ratio was at 67 and thus only marginally increased. There was no relevant proteinuria ([Table 1]).

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On the morning after hospitalization we decided to terminate the pregnancy, because of progressive liver failure with increasing coagulopathy. The patient delivered a healthy baby with normal APGAR and umbilical artery pH via cesarean section. The neonatologists examined the newborn and ruled out metabolic disorders (including long-chain 3-hydroxyacyl CoA dehydrogenase deficiency) by an extended screening.

The patient spent one night on the intensive care unit because of worsening coagulopathy and severe hyperbilirubinemia. She received 4g of fibrinogen. Her clinical situation improved significantly in the following days, with complete remission of the initial symptoms, decreasing aminotransferases and bilirubin and improving liver synthesis. Antibody testing showed no signs for autoimmune hepatitis. Testing for viral hepatitis and leptospira was negative ([Table 2]).

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There was a mild elevation in bile acids (30µmol/l) and the patient received an oral treatment with ursodeoxycholic acid. She was discharged six days after delivery and returned for follow-up-appointments and further diagnostics in the clinic for hepatology.

Discussion We diagnosed our patient with AFLP as she fulfilled eight of the 14 Swansea criteria suggested by Ch'ng et al. and presented severe coagulopathy with decreased antithrombin III, while HELLP remained a relevant differential diagnosis. All clinical symptoms and lab parameters for acute liver disease remitted quickly after termination of the pregnancy.

Diagnostic criteria for acute liver disease in pregnancy overlap and laboratory results may not be available quickly enough in a potentially life threatening situation for mother and child. Thus differential diagnosis can be a challenge and must sometimes be deferred until after delivery.

Publication History

Article published online:
15 November 2023