Seminars in Immunopathology

, Volume 31, Issue 3, pp 421–435 | Cite as

Autoimmune hepatitis

  • Diego Vergani
  • Maria Serena Longhi
  • Dimitrios P. Bogdanos
  • Yun Ma
  • Giorgina Mieli-Vergani
Review

Abstract

Autoimmune hepatitis (AIH) is an inflammatory liver disease affecting mainly females and characterised histologically by interface hepatitis, biochemically by elevated transaminase levels and serologically by the presence of autoantibodies and increased levels of immunoglobulin G. AIH responds to immunosuppressive treatment, which should be instituted as soon as diagnosis is made. Seropositivity for smooth muscle and/or anti-nuclear antibody defines type 1 AIH, while positivity for liver kidney microsomal type 1 antibody defines type 2 AIH. The aetiology of AIH is unknown, though both genetic and environmental factors are involved in its expression. Immune reactions against host liver antigens are believed to be the major mechanism of liver damage.

Keywords

Autoimmune hepatitis Liver Autoantibody Humoral immunity Cellular immunity Immune-regulation 

Abbreviations

AIH

autoimmune hepatitis

IAIHG

International Autoimmune Hepatitis Group

IgG

immunoglobulin G

ANA

anti-nuclear antibodies

SMA

anti-smooth muscle antibodies

LKM

liver kidney microsomal

anti-SLA/LP

anti-soluble liver antigen/liver pancreas antibody

LC1

liver cytosol type 1

LM

liver membrane

p-ANCA

perinuclear anti-neutrophil cytoplasmic antibodies

c-ANCA

cytoplasmic anti-neutrophil cytoplasmic antibodies

p-ANNA

perinuclear anti-nuclear neutrophil antibodies

AMA

anti-mitochondrial antibody

V

vessels

G

glomeruli

T

tubules

LSP

liver-specific protein

ASGPR

asialoglycoprotein receptor

ADH

alcohol dehydrogenase

APECED

autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy

tRNP(Ser)Sec

UGA tRNA suppressor associated antigenic protein

MHC

major histocompatibility complex

HLA

human leukocyte antigen

TCR

T-cell receptor

CTLA-4

cytotoxic T-lymphocyte associated antigen 4

NK

natural killer

APC

Antigen-presenting cell

TNF-α

Tumour necrosis factor alpha

TH

T-helper

Tr

T-regulatory

T-regs

CD4+ CD25+ regulatory T-cells

CTL

cytotoxic T lymphocyte

CYP2D6

cytochrome P450IID6

CTL

cytotoxic T-cells

IL

interleukin

IL2-R

IL-2 receptor

GITR

glucocorticoid inducible tumour necrosis factor receptor

FOXP3

forkhead winged helix transcription factor box P3

IFN-γ

interferon gamma

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Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  • Diego Vergani
    • 1
  • Maria Serena Longhi
    • 2
  • Dimitrios P. Bogdanos
    • 2
  • Yun Ma
    • 2
  • Giorgina Mieli-Vergani
    • 2
  1. 1.Institute of Liver Studies, Paediatric Liver CentreKing’s College HospitalLondonUK
  2. 2.King’s College London School of MedicineKing’s College HospitalLondonUK

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