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


CONCLUSIONS
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.
Institute of Liver Studies, Kings College Hospital, Denmark Hill, London SE5 9RS, United Kingdom.
Andrew D
AD
Yeoman
Institute of Liver Studies, Kings College Hospital, Denmark Hill, London SE5 9RS, United Kingdom.
Rachel H
RH
Westbrook
Institute of Liver Studies, Kings College Hospital, Denmark Hill, London SE5 9RS, United Kingdom.
Yoh
Y
Zen
Institute of Liver Studies, Kings College Hospital, Denmark Hill, London SE5 9RS, United Kingdom.
William
W
Bernal
Institute of Liver Studies, Kings College Hospital, Denmark Hill, London SE5 9RS, United Kingdom.
Thawab
T
Al-Chalabi
Institute of Liver Studies, Kings College Hospital, Denmark Hill, London SE5 9RS, United Kingdom.
Julia A
JA
Wendon
Institute of Liver Studies, Kings College Hospital, Denmark Hill, London SE5 9RS, United Kingdom.
John G
JG
O'Grady
Institute of Liver Studies, Kings College Hospital, Denmark Hill, London SE5 9RS, United Kingdom. Electronic address: michael.heneghan@nhs.net.
Michael A
MA
Heneghan
D000208:Acute Disease; D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000009:adverse effects; D000328:Adult; D061345:Early Medical Intervention / Q000379:methods; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage / Q000009:adverse effects; D006501:Hepatic Encephalopathy / Q000209:etiology / Q000517:prevention & control; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality / Q000503:physiopathology; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage / Q000009:adverse effects; D008111:Liver Function Tests / Q000379:methods; D016031:Liver Transplantation / Q000706:statistics & numerical data*; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D018805:Sepsis / Q000209:etiology / Q000517:prevention & control; D012698:Serologic Tests / Q000379:methods; D012720:Severity of Illness Index; D006113:United Kingdom / Q000453:epidemiology
D000305:Adrenal Cortex Hormones; D005938:Glucocorticoids; D007155:Immunologic Factors
Autoimmune hepatitis; Immunosuppression; Liver failure; Liver transplantation