Fulminant presentation of autoimmune hepatitis: clinical features and early predictors of corticosteroid treatment failure.
OBJECTIVE
Classical features of autoimmune hepatitis (AIH) may be altered during the abrupt onset of the disease. Corticosteroid therapy can be life-saving, but its use in the fulminant presentation of AIH (F-AIH) remains controversial. We aimed to assess the clinical features of patients with F-AIH and to describe the role of corticosteroids in this population.


METHODS
We retrospectively analyzed 154 adult patients with fulminant hepatic failure who were admitted to six liver transplantation (LT) programs. The AIH simplified criteria were used to identify patients with F-AIH.


RESULTS
We identified 40 (26%) patients with F-AIH. Compared with other etiologies, patients with F-AIH presented a longer interval from jaundice to encephalopathy (26 vs. 16 days, P=0.02) and a lower Model for End-Stage Liver Disease (MELD) score on admission (29 vs. 33, P=0.002). Overall, 25 (62%) patients with F-AIH underwent LT, eight (20%) patients survived, and seven (18%) died without LT. Seventeen patients received corticosteroids therapy, of whom seven (41%) survived without LT. Among the treated patients, higher MELD score and encephalopathy grade of 3 or more were associated significantly with corticosteroid failure.


CONCLUSIONS
Patients with F-AIH have a more indolent presentation compared with the non-F-AIH population. Altogether, only eight (20%) patients presenting with F-AIH survived without LT. A subset of patients with F-AIH and an initial MELD score less than 27 and low-grade hepatic encephalopathy might benefit from administration of corticosteroids.
aHepatology and Liver Transplant Unit bDepartment of Medicine, Hospital Universitario Austral cLiver Unit, Hospital Italiano de Buenos Aires dHepatology and Liver Transplant Unit, Fundación Favaloro eHepatology and Liver Transplant Unit, Hospital Alemán, Buenos Aires fLiver Transplant Unit, Sanatorio Allende gHepatology and Liver Transplant Unit, Hospital Privado de Córdoba, Córdoba, Argentina.
Manuel
M
Mendizabal
Sebastián
S
Marciano
María G
MG
Videla
Margarita
M
Anders
Alina
A
Zerega
Domingo C
DC
Balderramo
Matías R
MR
Tisi Baña
Martín
M
Barrabino
Octavio
O
Gil
Ricardo
R
Mastai
Silvina
S
Yantorno
Adrián
A
Gadano
Marcelo O
MO
Silva
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D005165:Factor V / Q000378:metabolism; D005260:Female; D006501:Hepatic Encephalopathy / Q000097:blood / Q000209:etiology*; D019693:Hepatitis, Autoimmune / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D019934:International Normalized Ratio; D017114:Liver Failure, Acute / Q000097:blood / Q000209:etiology*; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000031:analogs & derivatives* / Q000627:therapeutic use; D012189:Retrospective Studies; D012720:Severity of Illness Index; D015996:Survival Rate; D017211:Treatment Failure
D000893:Anti-Inflammatory Agents; C008352:meprednisone; D005165:Factor V; D011241:Prednisone