What are the symptoms of acute fatty liver disease?

Symptoms of Acute Fatty Liver of Pregnancy (AFLP)

The most common presenting symptoms of acute fatty liver of pregnancy include nausea/vomiting (88.5%), jaundice (71.4%), and abdominal pain (51.4%), forming what has been described as the "AFLP-Triad" of symptoms. 1

Clinical Presentation

Acute fatty liver of pregnancy typically occurs in the third trimester or early postpartum period, though rare cases have been reported as early as the second trimester. The symptom profile includes:

Primary Symptoms

• Nausea and vomiting (88.5%)
• Jaundice (71.4%)
• Abdominal pain, often epigastric (51.4%)
• Malaise
• Headache
• Features of pre-eclampsia (in approximately 50% of cases)
• Polydipsia/polyuria

Advanced Symptoms (indicating disease progression)

• Encephalopathy (40%)
• Hypoglycemia (94.2%)
• Signs of coagulopathy (77.1%)
• Ascites (48.5%)

Laboratory Findings

The diagnosis of AFLP is supported by the Swansea criteria, which requires the presence of at least 6 of the following findings 2:

• Elevated transaminases
• Elevated bilirubin
• Hypoglycemia (very common, seen in 94.2% of cases)
• Leukocytosis
• Elevated uric acid
• Elevated ammonia
• Renal impairment (seen in 94.2% of cases)
• Coagulopathy (abnormal PT/PTT, low fibrinogen)

Imaging Findings

• Ultrasound may show ascites or bright liver
• CT scan may reveal decreased hepatic density

Important Clinical Considerations

1. Timing: While AFLP typically occurs in the third trimester, there are documented cases as early as 22-26 weeks gestation 3, 4. This unusual early presentation should not exclude the diagnosis if other symptoms are present.

2. Differential Diagnosis: AFLP can be difficult to distinguish from HELLP syndrome, as approximately 50% of AFLP patients have features of pre-eclampsia 2.

3. Disease Progression: AFLP can rapidly progress to acute liver failure with encephalopathy, renal failure, and coagulopathy if not promptly recognized and treated 5.

4. Common Pitfalls:

   • Misdiagnosis as viral hepatitis or cholecystitis
   • Delayed recognition due to nonspecific initial symptoms
   • Failure to consider AFLP in second trimester presentations

5. Warning Signs of Severe Disease:

   • Encephalopathy
   • Elevated serum lactate
   • MELD score >30
   • Swansea criteria >7
   • These patients require intensive care 2

Diagnostic Algorithm

1. Consider AFLP in any pregnant woman (especially in third trimester) presenting with:

   • Nausea/vomiting
   • Jaundice
   • Abdominal pain

2. Immediately order:

   • Liver function tests
   • Complete blood count
   • Coagulation profile
   • Renal function tests
   • Blood glucose
   • Uric acid

3. Apply Swansea criteria (≥6 findings strongly correlates with AFLP diagnosis)

4. Obtain abdominal ultrasound to assess for ascites or bright liver

5. Consider liver biopsy only in atypical cases where diagnosis remains uncertain

Early recognition of this constellation of symptoms is critical, as prompt delivery is the definitive treatment and has reduced maternal mortality from historical rates of up to 92% to less than 10% in recent years 2.