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The Management of Autoimmune Hepatitis Patients with Decompensated Cirrhosis: Real-World Experience and a Comprehensive Review

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Abstract

There is a paucity of information related to the usefulness of corticosteroid therapy in autoimmune hepatitis (AIH) with decompensated cirrhosis. In this study, we sought to determine the therapeutic effect of corticosteroids in this special group of AIH patients. Eighty-two AIH patients with decompensated cirrhosis were included through a retrospective analysis from January 2009 to September 2015. Sixty-four patients were treated with corticosteroids while 18 patients did not receive any corticosteroids. Clinical, laboratory, and histological characteristics and outcomes were analyzed comparing corticosteroid-treated and untreated groups. Patients that did not receive corticosteroids were older than corticosteroid-treated patients and had a worse survival. In corticosteroid-treated group, 40 of 64 patients reverted to compensated state and 15 patients remained decompensated, while 9 patients experienced liver-related death or transplantation. Patients who reverted to compensated state had significantly greater ALT, AST, GGT, white blood cell count, and platelet levels at presentation. Changes (Δ) in total bilirubin (TBIL) and MELD scores at day 7 after starting corticosteroid therapy revealed favorable predictive effects of treatment outcomes. Survival was significantly greater in patients with a ΔTBIL <−0.196 mg/dL (p = 0.001) 7 days after treatment. Infection was the most common cause of death or transplantation in the patients with treatment failure. Although it cannot be determined whether the results were due to the therapy or underlying patient characteristics, survival was greater in the corticosteroid-treated group with the benefit being greatest in patients with the greatest decrease in TBIL at day 7 after starting corticosteroid therapy.

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Abbreviations

AIH:

Autoimmune hepatitis

ALP:

Alkaline phosphatase

ALT:

Alanine aminotransferase

AST:

Aspartate aminotransferase

AZA:

Azathioprine

GGT:

Gamma-glutamyltranspeptidase

Hb:

Hemoglobin

HE:

Hepatic encephalopathy

IgG:

Immunoglobulin G

iMELD:

The Integrated Model For End-Stage Liver Disease

INR:

International normalized ratio

LT:

Liver transplantation

MELD:

Model for end-stage liver disease

MELD-Na:

MELD with the Incorporation of Serum Sodium

MESO:

Model for End-Stage Liver Disease to sodium

MMF:

Mycophenolate mofetil

PBC:

Primary biliary cholangitis

PLT:

Platelet

SBP:

Spontaneous bacterial peritonitis

TBIL:

Total bilirubin

UDCA:

Ursodeoxycholic acid

WBC:

White blood cells

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Authors Contributions

Zhaoyue Wang, Li Sheng, Yue Yang: data acquisition; data analysis; manuscript drafting

Fan Yang, Xiao Xiao: data acquisition; data analysis

Yiran Wei, Jun Zhang, Yanmei Li: data acquisition

Qi Miao: histological analysis support

Jing Hua, Canjie Guo, Ruqi Tang: manuscript editing

Jingyuan Fang, Dekai Qiu: study concept and design; study supervision

Edward L Krawitt, Christopher L. Bowlus: critical revision of the manuscript for important intellectual content

M. Eric Gershwin: critical revision of the manuscript for important intellectual content; approval of the final version of the manuscript

Xiong Ma, Qixia Wang: study concept and design; study supervision; approval of the final version of the manuscript

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Authors and Affiliations

  1. State Key Laboratory for Oncogenes and Related Genes, Key Laboratory of Gastroenterology and Hepatology, Ministry of Health, Division of Gastroenterology and Hepatology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai Cancer Institute, Shanghai Institute of Digestive Disease, 145 Middle Shandong Road, Shanghai, 200001, China

    Zhaoyue Wang, Li Sheng, Yue Yang, Fan Yang, Xiao Xiao, Jing Hua, Canjie Guo, Yiran Wei, Ruqi Tang, Qi Miao, Jun Zhang, Yanmei Li, Jingyuan Fang, Dekai Qiu, Qixia Wang & Xiong Ma

  2. Department of Medicine, University of Vermont, Burlington, VT, USA

    Edward L Krawitt

  3. Department of Medicine, Dartmouth College, Hanover, NH, USA

    Edward L Krawitt

  4. Division of Gastroenterology and Hepatology, University of California Davis School of Medicine, Davis, USA

    Christopher L. Bowlus

  5. Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, Genome and Biomedical Sciences Facility, Davis, USA

    M. Eric Gershwin

Authors
  1. Zhaoyue Wang
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  2. Li Sheng
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  3. Yue Yang
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  4. Fan Yang
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  7. Canjie Guo
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  8. Yiran Wei
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  9. Ruqi Tang
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  10. Qi Miao
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  11. Jun Zhang
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  12. Yanmei Li
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  13. Jingyuan Fang
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  14. Dekai Qiu
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  15. Edward L Krawitt
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  16. Christopher L. Bowlus
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  17. M. Eric Gershwin
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  18. Qixia Wang
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  19. Xiong Ma
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Corresponding authors

Correspondence to M. Eric Gershwin, Qixia Wang or Xiong Ma.

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Conflict of Interest

The authors declare no financial conflict of interest exists.

Financial Support

This work was supported by awards from the National Natural Science Foundation of China (# 81325002 to Xiong Ma, #81570511 to Qixia Wang,#81421001 to Jingyuan Fang, #81400608 to Ruqi Tang).

Additional information

Zhaoyue Wang, Li Sheng and Yue Yang are co-first authors.

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Wang, Z., Sheng, L., Yang, Y. et al. The Management of Autoimmune Hepatitis Patients with Decompensated Cirrhosis: Real-World Experience and a Comprehensive Review. Clinic Rev Allerg Immunol 52, 424–435 (2017). https://doi.org/10.1007/s12016-016-8583-2

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  • DOI: https://doi.org/10.1007/s12016-016-8583-2

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