Treatment of Autoimmune Hepatitis with Acute Liver Failure and Hepatic Encephalopathy

Acute severe autoimmune hepatitis (AIH) that progresses to acute liver failure — marked by INR >2 and hepatic encephalopathy — or to acute-on-chronic liver failure (ACLF) demands urgent, structured clinical decision-making. Available evidence in this subset is limited, and outcomes without timely intervention are poor.

Clinical Scenario

This protocol applies to patients with acute severe AIH presenting with acute liver failure (INR >2 and hepatic encephalopathy) or acute-on-chronic liver failure (ACLF). The combination of coagulopathy and encephalopathy defines a high-acuity population in which the key clinical challenge is whether and when to initiate specific treatment, and whether transplantation must be pursued in parallel.

Treatment Approach

The primary step in this setting is direct evaluation for liver transplantation, including prompt discussion with a transplant centre. When pharmacological treatment is considered, it involves close monitoring of treatment efficacy and strict surveillance for infectious complications. The complete decision pathway, agent selection, and monitoring parameters are detailed in the full structured protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/j.jhep.2025.03.017

  1. Direct evaluation (discussion with a LT centre) for LT is recommended in patients with acute severe AIH with ALF or ACLF, as data on the role of corticosteroids in these patients is very limited and outcomes are poor (LoE 3, strong recommendation, strong consensus).
  2. However, managing patients with acute severe AIH (icteric, INR > −1.5 but <2, without hepatic encephalopathy and without chronic lesions on liver biopsy), with ALF (INR >2 and hepatic encephalopathy) or ACLF, can be challenging, and the most crucial decision is whether and when to initiate corticosteroid treatment.
  3. If corticosteroids are given to patients with acute severe AIH with ALF or ACLF, strict surveillance for infections and close monitoring of their efficacy is recommended (LoE 2, strong recommendation, strong consensus).
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