Prognosis of acute severe autoimmune hepatitis (AS-AIH): the role of corticosteroids in modifying outcome

J Hepatol. 2014 Oct;61(4):876-82. doi: 10.1016/j.jhep.2014.05.021. Epub 2014 May 16.


Background & aims: No standardised definition exists for acute, severe AIH (AS-AIH). However, rapid identification of AS-AIH and early corticosteroid therapy may prevent the need for liver transplantation (LT). We set out to determine the clinical outcomes of patients with AS-AIH presenting to our institution with particular focus on the role of corticosteroids.

Methods: Retrospective analysis of a prospectively collated database identified patients presenting with AS-AIH from 1999 to 2009. We defined AS-AIH as an acute presentation with an INR of ⩾1.5 at any time without histological evidence of cirrhosis.

Results: 32 patients were identified with AS-AIH. Among the 32 AS-AIH patients 23 were treated with corticosteroids of whom 10 (48%) required LT, whilst all 9 untreated patients required LT (p = 0.01). Untreated patients demonstrated higher MELD scores at presentation (34 vs. 28 p = 0.01) and a non-significant decrease in episodes of sepsis but no difference in sepsis or mortality was observed between untreated or treated patients (11% vs. 26% p = 0.6 and 22% vs. 17% p = 0.99 respectively). Among treated patients, no difference in MELD scores was observed between responders or failures. Despite 59% undergoing LT, six deaths (19%) occurred.

Conclusion: In a well characterised cohort of patients with AS-AIH, almost 60% required LT and 20% died. There was no difference in prognostic scores between steroid responders and failures and steroid exposure did not appear to jeopardise survival. Patients with AS-AIH should be considered for a trial of corticosteroids expediently whilst a thorough search for sepsis and assessment for LT should occur if clinical deterioration or encephalopathy develops.

Keywords: Autoimmune hepatitis; Immunosuppression; Liver failure; Liver transplantation.

MeSH terms

  • Acute Disease
  • Adrenal Cortex Hormones* / administration & dosage
  • Adrenal Cortex Hormones* / adverse effects
  • Adult
  • Early Medical Intervention / methods
  • Female
  • Glucocorticoids / administration & dosage
  • Glucocorticoids / adverse effects
  • Hepatic Encephalopathy* / etiology
  • Hepatic Encephalopathy* / prevention & control
  • Hepatitis, Autoimmune* / complications
  • Hepatitis, Autoimmune* / diagnosis
  • Hepatitis, Autoimmune* / drug therapy
  • Hepatitis, Autoimmune* / mortality
  • Hepatitis, Autoimmune* / physiopathology
  • Humans
  • Immunologic Factors / administration & dosage
  • Immunologic Factors / adverse effects
  • Liver Function Tests / methods
  • Liver Transplantation / statistics & numerical data*
  • Male
  • Prognosis
  • Retrospective Studies
  • Sepsis* / etiology
  • Sepsis* / prevention & control
  • Serologic Tests / methods
  • Severity of Illness Index
  • United Kingdom / epidemiology


  • Adrenal Cortex Hormones
  • Glucocorticoids
  • Immunologic Factors