Environmental factors in primary biliary cirrhosis

Carlo Selmi

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease more commonly found in women (with most cases being diagnosed in the fourth to fifth decades of life) that share peculiar features. Among these, serum antimitochondrial autoantibodies (AMA), serum hyper-immunoglobulin M, and progressive destruction of intrahepatic bile ducts are the most common clinical findings. The pathogenesis of the selective destruction of small-duct biliary epithelial cells is still unknown, although an autoimmune mechanism appears likely due to the detection of autoreactive T and B cells. Genetic background is critical in determining susceptibility to the disease, despite the limited associations with alleles within the major histocompatibility complex. However, among other observations, incomplete concordance of the disease in monozygotic twins and epidemiology strongly support a role for environmental factors in PBC onset. Molecular mimicry by either infectious agents or chemicals has been proposed to induce breakdown of immune tolerance in genetically predisposed individuals. This hypothesis is further supported by clinical (risk factors) and experimental evidence obtained also in animal models.

Original languageEnglish (US)
JournalHepatology Research
Volume37
Issue numberSUPPL. 3
DOIs
StatePublished - Oct 2007

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Biliary Liver Cirrhosis
Diseases in Twins
Molecular Mimicry
Intrahepatic Bile Ducts
Immune Tolerance
Monozygotic Twins
Disease Susceptibility
Major Histocompatibility Complex
Serum
Autoantibodies
Immunoglobulin M
Liver Diseases
Epidemiology
B-Lymphocytes
Animal Models
Epithelial Cells
Alleles
T-Lymphocytes
Genetic Background

Keywords

  • Autoimmune cholangitis
  • Geoepidemiology
  • Molecular mimicry
  • Xenobiotics

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Environmental factors in primary biliary cirrhosis. / Selmi, Carlo.

In: Hepatology Research, Vol. 37, No. SUPPL. 3, 10.2007.

Research output: Contribution to journalArticle

Selmi, Carlo. / Environmental factors in primary biliary cirrhosis. In: Hepatology Research. 2007 ; Vol. 37, No. SUPPL. 3.
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