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Autoimmune hepatitis

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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.

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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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Authors and Affiliations

  1. Institute of Liver Studies, Paediatric Liver Centre, King’s College Hospital, Denmark Hill, London, SE5 9RS, UK

    Diego Vergani

  2. King’s College London School of Medicine, King’s College Hospital, Denmark Hill, London, SE5 9RS, UK

    Maria Serena Longhi, Dimitrios P. Bogdanos, Yun Ma & Giorgina Mieli-Vergani

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  1. Diego Vergani
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  2. Maria Serena Longhi
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  3. Dimitrios P. Bogdanos
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  4. Yun Ma
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  5. Giorgina Mieli-Vergani
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Correspondence to Diego Vergani.

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Vergani, D., Longhi, M.S., Bogdanos, D.P. et al. Autoimmune hepatitis. Semin Immunopathol 31, 421–435 (2009). https://doi.org/10.1007/s00281-009-0170-7

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