Introduction:

Autoimmune hepatitis can initially manifest itself in various clinical ways. Its acute presentation with increased levels of bilirubin, fall of biosynthetic function and massive/submassive necrosis or cirrhosis in histology, is a marker of severity of it. The decision to start corticosteroid treatment in this scenario is a challenge in clinical practice. The objectives of this work were to assess the degree of response to treatment, response predictors, and overall and transplant-free survival in patients whose clinical debut was acute and severe.

Results:

33 patients with total bilirubin greater than 2.5 mg/dL, without prior (naive) treatment, who met diagnostic criteria according to the international autoimmune hepatitis group, were included. 97% were women with a median age of 52, 58% were in the cirrhosis stage with an average MELD of 24, and 15% had massive/submassive necrosis in the liver biopsy sample. In 27 cases treatment with cortiocosteroids was initiated, 66.7% evolved with complete remission, 14.8% with partial remission, and in 18.5% there was failure of corticosteroid treatment. Basal cholesterol and the presence of hepatic encephalopathy were predictors of non-response to corticosteroid treatment, while pre-treatment MELD and basal cholesterol were the statistically significant variables associated with liver transplant rescue failure. In patients treated with corticosteroids, overall and transplant-free survival at 5 years based on MELD (<25 vs ≥ 25) was 90% vs 60% respectively.

Conclusion:

The high survival rates achieved under medical treatment that were demonstrated in this series reaffirm the need to prioritize the use of corticosteroids in patients with acute and severe autoimmune hepatitis.

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