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Liver Immunology pp 253–279Cite as

Primary Biliary Cirrhosis

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Abstract

PBC is a chronic cholestatic liver disease characterized by high-titer serum antimitochondrial autoantibodies (AMAs) and autoimmune-mediated destruction of small- and medium-sized intrahepatic bile ducts [1–3]. The first description of biliary cirrhosis, albeit possibly secondary, can be traced back to the work of the Italian pathologist Giovanni Battista Morgagni from Padua in 1761; the first report of nonobstructive biliary cirrhosis was by Addison and Gull in 1851. Subsequently, the term PBC was accepted in the medical literature [4], and in 1959 Dame Sheila Sherlock described the first series of patients affected by PBC who had been followed over the previous decade and noted that patients presented with pruritus as well as the signs and symptoms of end-stage liver disease including jaundice [5].

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Abbreviations

2-OADC:

2-oxo acid dehydrogenase complex

ALP:

Alkaline phosphatase

AMAs:

Antimitochondrial autoantibodies

ANAs:

Antinuclear antibodies

AST:

Aspartate transaminase

α-GalCer:

α-Galactosylceramide

BCOADC:

Branched-chain 2-oxo acid dehydrogenase complex

BECs:

Biliary epithelial cells

CA:

Cholangitis activity

CTLA-4:

Cytotoxic T lymphocyte antigen-4

E3BP:

Dihydrolipoamide dehydrogenase (E3)-binding protein

ELISA:

Enzyme-linked immunosorbent assay

GWAS:

Genome-wide association studies

HA:

Hepatitis activity

HCC:

Hepatocellular carcinoma

HLA:

Human leukocyte antigen

ICAM-1:

Intercellular adhesion molecule 1

IL:

Interleukin

IP:

Intraperitoneally

IRAK-M:

Interleukin-1 receptor-associated kinase M

LFA-3:

Lymphocyte-associated antigen 3

MCP-1:

Monocyte chemotactic protein-1

MHC:

Major histocompatibility complex

NF-kB:

NF kappa B

NKT:

Natural killer T

OGDC:

Oxoglutarate dehydrogenase complex

OLT:

Orthotopic liver transplantation

PBC:

Primary biliary cirrhosis

PD:

Programmed death

PDC:

Pyruvate dehydrogenase complex

QSAR:

Quantitative structure–activity relationship

SNPs:

Single-nucleotide polymorphisms

STAT4:

Signal transducer and activator of transcription 4

TGF:

Transforming growth factor

TLRs:

Toll-like receptors

TNF:

Tumor necrosis factor

TRAIL :

TNF-related apoptosis-inducing ligand

Treg:

Regulatory T cells

UDCA:

Ursodeoxycholic acid

VCAM-1:

Vascular cell adhesion molecule-1

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Authors and Affiliations

  1. Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, 451 Health Sciences Drive, Suite 6510, Davis, CA, 95616, USA

    Pietro Invernizzi, Patrick S. C. Leung & M. Eric Gershwin

  2. Division of Gastroenterology and Hepatology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai Institute of Digestive Disease, Shanghai, China

    Xiong Ma

  3. Key Laboratory of Gastroenterology and Hepatology, Ministry of Health (Shanghai Jiao-Tong University), Shanghai, China

    Xiong Ma

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  1. Pietro Invernizzi
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  2. Patrick S. C. Leung
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  3. Xiong Ma
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  4. M. Eric Gershwin
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Correspondence to M. Eric Gershwin .

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  1. Division of Rheumatology, Allergy and Clinical Immunology, University of California School of Medicine, Davis, California, USA

    M. Eric Gershwin

  2. Medicine and Surgery, Baylor College of Medicine, Houston, Texas, USA

    John M. Vierling

  3. Department of Gastroenterology, Hepatology and Endocrinology, Medical School of Hannover, Germany

    Michael P. Manns

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Invernizzi, P., Leung, P.S.C., Ma, X., Gershwin, M.E. (2014). Primary Biliary Cirrhosis. In: Gershwin, M., Vierling, J., Manns, M. (eds) Liver Immunology. Springer, Cham. https://doi.org/10.1007/978-3-319-02096-9_18

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