Acute fatty liver of pregnancy and other liver disorders in pregnancy

It is 34 weeks and the registrar on the antenatal ward has asked you to see a primigravida with two days of vomiting and right-upper-quadrant pain. She looks unwell; her bloods come back with a glucose of 2.1 mmol/L, an INR of 1.9 and an ALT of 240 IU/L. The midwife's note says "preeclampsia — check LFTs". You are about to make the most important diagnosis of this pregnancy. AFLP is rare, but the Swansea criteria exist precisely for this moment, and the treatment is unambiguous: deliver. This topic is that decision.^[2][11]

Overview and definition

Liver disease in pregnancy splits cleanly into two questions. The first — is it caused by the pregnancy? — directs everything that follows. The second — is it AFLP, HELLP, preeclampsia, cholestasis, or something coincidental? — directs today's management.^[8][9]

Pregnancy-specific liver disorders arise from the placenta-hormone-fetus interaction and resolve after delivery:^[8]

• AFLP — microvesicular fatty infiltration of hepatocytes causing acute liver failure; third trimester; thecatastrophic end of the spectrum.
• HELLP syndrome — haemolysis, elevated liver enzymes, low platelets; a variant of severe preeclampsia; covered in its own topic.
• Preeclampsia with hepatic involvement — periportal haemorrhage and infarction; hypertension and proteinuria are the core features.
• Intrahepatic cholestasis of pregnancy — pruritus and elevated bile acids; covered in its own topic.
• Hyperemesis gravidarum with liver dysfunction — first-trimester dysfunction that resolves with hydration.^[8]

Coincidental and pre-existing liver disease behave in pregnancy as they would outside it, but pregnancy can flare, transmit, or unmask them:^[8]

• Viral hepatitis — hepatitis B and C with vertical-transmission considerations; hepatitis E with high maternal mortality; herpes simplex hepatitis in the immunocompromised.
• Autoimmune hepatitis — flares during pregnancy and the postpartum; immunosuppression must continue.
• Gallstones — the most common surgical cause of jaundice in pregnancy; conservative first.
• Cirrhosis and portal hypertension — uncommon but dangerous; variceal bleeding risk peaks in the second trimester.^[8]

The single sentence that frames the topic: AFLP is the obstetric emergency in this group, the Swansea criteria are how you spot it, and delivery is the treatment.^[1][4]

References13ShowHide

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2. [2]Knight M, Nelson-Piercy C, Kurinczuk JJ, Spark P, Brocklehurst P; UK Obstetric Surveillance System A prospective national study of acute fatty liver of pregnancy in the UK Gut, 2008.PMID 18332072
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11. [11]Martin JN Jr, Tucker JM Missing or making the timely diagnosis of acute fatty liver of pregnancy (AFLP): lessons learned J Matern Fetal Neonatal Med, 2022.PMID 33032483
12. [12]Terrault NA, Lok ASF, McMahon BJ, Chang KM, Hwang JP, Jonas MM, Brown RS Jr, Bzowej NH, Wong JB Update on prevention, diagnosis, and treatment of chronic hepatitis B: AASLD 2018 hepatitis B guidance Hepatology, 2018.PMID 29405329
13. [13]Westbrook RH, Yeoman AD, Kriese S, Heneghan MA Outcomes of pregnancy in women with autoimmune hepatitis J Autoimmun, 2012.PMID 22261501