Retrospective Study
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World J Gastroenterol. Jun 21, 2014; 20(23): 7473-7479
Published online Jun 21, 2014. doi: 10.3748/wjg.v20.i23.7473
Clinical characteristics and corticosteroid therapy in patients with autoimmune-hepatitis-induced liver failure
Bing Zhu, Shao-Li You, Zhi-Hong Wan, Hong-Ling Liu, Yi-Hui Rong, Hong Zang, Shao-Jie Xin
Bing Zhu, Shao-Li You, Zhi-Hong Wan, Hong-Ling Liu, Yi-Hui Rong, Hong Zang, Shao-Jie Xin, Liver Failure Treatment and Research Center, 302nd Military Hospital, Beijing 100039, China
Author contributions: Zhu B, You SL and Wan ZH contributed equally to this work; Zhu B, You SL, Wan ZH and Xin SJ designed research; Zhu B, You SL, Liu HL, Rong YH and Zang H performed the research; Zhu B, You SL and Wan ZH analyzed the data and wrote the paper; all authors have read and approved the final manuscript.
Supported by Grants from the 12th Five-Year National Science and Technology Major Project for Infectious Diseases, No. 2012ZX10002004-005; 12th Five-Year Major Project of PLA, No. BWS11J075
Correspondence to: Shao-Jie Xin, Professor, Liver Failure Treatment and Research Center, 302nd Military Hospital, No. 100, Middle of West 4th Ring RD, Beijing 100039, China. xinshaojie302@163.com
Telephone: +86-10-66933433 Fax: +86-10-66933434
Received: December 3, 2013
Revised: February 25, 2014
Accepted: April 15, 2014
Published online: June 21, 2014
Abstract

AIM: To investigate the clinical features, response to corticosteroids, and prognosis of autoimmune hepatitis (AIH)-induced liver failure in China.

METHODS: A total of 22 patients (19 female and 3 male; average age 51 ± 15 years) with AIH-induced liver failure treated in our hospital from 2004 to 2012 were retrospectively analyzed. Clinical, biochemical and pathological characteristics of the 22 patients and responses to corticosteroid treatment in seven patients were examined retrospectively. The patients were divided into survivor and non-survivor groups, and the clinical characteristics and prognosis were compared between the two groups. The t test was used for data analysis of all categorical variables, and overall survival was calculated by the Kaplan-Meier method.

RESULTS: At the time of diagnosis, mean IgG was 2473 ± 983 mg/dL, with three (18.8%) patients showing normal levels. All of the patients had elevated serum levels of antinuclear antibody (≥ 1:640). Liver histology from one patient showed diagnostic pathological changes, including massive necrosis and plasma cell infiltration. Four patients survived (18.2%) and 18 died (81.8%) without liver transplantation. The results showed that patients with low admission Model for End-Stage Liver Disease (MELD) scores (21.50 ± 2.08 vs 30.61 ± 6.70, P < 0.05) and corticosteroid therapy (100% vs 16.7%, P < 0.05) had better prognosis. A total of seven patients received corticosteroid therapy, of whom, four responded and survived, and the other three died. Survivors showed young age, shorter duration from diagnosis to corticosteroid therapy, low MELD score, and absence of hepatic encephalopathy at the time of corticosteroid administration. Six patients who were administered corticosteroids acquired fungal infections but recovered after antifungal therapy.

CONCLUSION: Early diagnosis and corticosteroid therapy are essential for improving the prognosis of patients with AIH-induced liver failure without liver transplantation.

Keywords: Autoimmune hepatitis, Liver failure, Autoantibody, Prognosis, Corticosteroid therapy

Core tip: We describe the clinical characteristics and prognosis of autoimmune-hepatitis-induced liver failure in China. Early diagnosis and initiating corticosteroid therapy at an early stage may be essential for improving the prognosis of patients with AIH-induced liver failure without liver transplantation.