ALF

1. Fulminant hepatic failure in
AIH

2. Objectives
• Definition of Fulminant hepatic failure
• Acute/ Sub-acute
• Criteria
• Steroid Role
• Immune Modulator Role

3. • Fulminant hepatic failure (FHF) is defined as severe acute liver
failure in a patient with no preexisting liver disease, with
encephalopathy developing within 2 weeks of the first
manifestation of liver disease.

6. Autoimmune Hepatitis
• AIH usually results in liver injury as a consequence of chronic hepatitis
and cirrhosis. However, rarely 2-5%, patients may present with
fulminant liver failure.

8. ALF
Pediatric acute liver failure (PALF) can be defined as:
• Biochemical evidence of acute liver injury in a child with no known
evidence of chronic liver disease along with at least one of the
following:
• INR > 1.5, not corrected with vitamin K supplementation, with
encephalopathy.

10. Steroid
• There are no strong data to suggest that an intravenous route is
superior to an oral route. There are considerable variations in clinical
practice throughout the world. Interestingly, recent multicenter data
suggest that low-dose corticosteroids (<0.5 mg/kg/day) provide
similar response rates in the normalization of transaminases when
compared with high-dose corticosteroids (≥0.5 mg/kg/day), albeit in a
group of patients with AIH as opposed to AS-AIH.

11. Role of Other Immunosuppressants
• There is no recognized role for alternative immunomodulators in AS-
AIH. In an acute presentation of AIH, it is not recommended to start
azathioprine for a number of reasons including poor drug metabolism
and risk of worsening cholestasis. MMF and calcineurin inhibitors
have only been shown to be effective in small observational studies.

12. GUIDELINE RECOMMENDATIONS
• In children or adults with AIH who have treatment failure, incomplete
response, or drug intolerance to first-line agents, the AASLD suggests
the use of MMF or TAC to achieve and maintain biochemical
remission (conditional recommendation, low certainty).
• Based on a superior ease of use and side-effect profile, the AASLD
suggests a trial of MMF over TAC as the initial second-line agent in
patients with AIH (conditional recommendation, very low certainty).