O-32 FULMINANT AUTOIMMUNE HEPATITIS: CLINICAL PRESENTATION, OUTCOME AND PROGNOSTIC FACTORS.

Introduction and Objectives

Early identification of fulminant autoimmune hepatitis could be lifesaving or prevent liver transplantation, but rapid diagnostic and prognostic criteria are lacking. This study aimed to assess the clinical features and outcomes of fulminant AIH. –To analyze prognostic factors related to poor outcomes (requirement of transplantation or death).

Materials and Methods

We retrospectively reviewed 307 consecutive patients evaluated for fulminant hepatic failure (1994-June 2020) in our Unit. Patient work-up consisted of viral serologies, autoantibodies, gammaglobulin, drug screening and ceruloplasmin. Since 2003, selected hemodynamically and neurologically stable patients have received a transjugular liver biopsy.

Results

86 patients (28,01%) fulfilled the criteria for fulminant AIH (AIH simplified criteria). Seven were excluded from analysis due to cirrhosis Oral meprednisone 60 mg or via nasogastric tube was started at diagnosis in 67 patients until death, transplantation, recovery or futility. Biochemical and clinical variables were analyzed. One patient developed hyperacute encephalopathy, 33 within 7/28 days post jaundice (41.7 %) and 45 (55.9 %) subacute encephalopathy (>28 days). 63/79 patients died or required liver transplantation (median time 7.8 days,1-34 days). 48 (60 %) patients underwent LT, 16 (20%) patients survived, and 16 (20 %) died without LT. Seven transplanted patients died early post OLT (infectious n=5, neurological complications n=2). Variables associated with bad prognosis were: prothrombin time < 20% or grade IV encephalopathy at steroid initiation, LC+ or LKM-1 +, massive necrosis, no >20% improvement of prothrombin time by day three post-steroids (p<0.05). Patients diagnosed before 2003 had the worst prognosis (87 vs. 71%), probably related to the shorter time to diagnosis since the introduction of biopsy (2.1±1.7 days vs. 4.6±2.1 days, p<0.05). Among patients who recovered, 5/16 were weaned from immunosuppression at a median of 4.5 years of treatment without relapse.

Conclusions

The disease course is aggressive, with death or requirement of liver transplantation in 80 % of patients. Early diagnosis and treatment may improve survival.