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Springer Seminars in Immunopathology : Volume 39

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Old and rising stars in the lymphoid liver

Primary biliary cirrhosis

Lymphocyte recruitment and homing to the liver in primary biliary cirrhosis and primary sclerosing cholangitis

The immunopathology of human biliary cell epithelium

Microanatomy of the liver immune system

Mononuclear cells in liver fibrosis

The immunopathogenesis of alcoholic and nonalcoholic steatohepatitis: two triggers for one disease?

Biliary atresia

The immunobiology of primary sclerosing cholangitis

Liver architecture, cell function, and disease

Contribution of human hematopoietic stem cells to liver repair

Autoimmune hepatitis

Springer Seminars in Immunopathology : Volume 31, Issue 2, July 2009

Springer Seminars in Immunopathology : Volume 31, Issue 1, June 2009

Springer Seminars in Immunopathology : Volume 30

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The immunobiology of primary sclerosing cholangitis

Content Provider	Springer Nature Link
Author	Aron, Jonathan H. Bowlus, Christopher L.
Copyright Year	2009
Abstract	Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease histologically characterized by the presence of intrahepatic and/or extrahepatic biliary duct concentric, obliterative fibrosis, eventually leading to cirrhosis. Approximately 75% of patients with PSC have inflammatory bowel disease. The male predominance of PSC, the lack of a defined, pathogenic autoantigen, and the potential role of the innate immune system suggest that it may be due to dysregulation of immunity rather than a classic autoimmune disease. However, PSC is associated with several classic autoimmune diseases, and the strongest genetic link to PSC identified to date is with the human leukocyte antigen DRB01*03 haplotype. The precise immunopathogenesis of PSC is largely unknown but likely involves activation of the innate immune system by bacterial components delivered to the liver via the portal vein. Induction of adhesion molecules and chemokines leads to the recruitment of intestinal lymphocytes. Bile duct injury results from the sustained inflammation and production of inflammatory cytokines. Biliary strictures may cause further damage as a result of bile stasis and recurrent secondary bacterial cholangitis. Currently, there is no effective therapy for PSC and developing a rational therapeutic strategy demands a better understanding of the disease.
Starting Page	383
Ending Page	397
Page Count	15
File Format	PDF
ISSN	18632297
Journal	Springer Seminars in Immunopathology
Volume Number	31
Issue Number	3
e-ISSN	18632300
Language	English
Publisher	Springer-Verlag
Publisher Date	2009-05-26
Publisher Place	Berlin, Heidelberg
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	sclerosing cholangitis Biliary cirrhosis Pathogenesis Inflammatory bowel disease Internal Medicine Immunology
Content Type	Text
Resource Type	Article
Subject	Immunology and Allergy Immunology

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Immunomodulatory Effect of Vancomycin on Treg in Pediatric Inflammatory Bowel Disease and Primary Sclerosing Cholangitis

Lymphocyte recruitment and homing to the liver in primary biliary cirrhosis and primary sclerosing cholangitis

Animal models in primary biliary cirrhosis and primary sclerosing cholangitis.

The immunophysiology and apoptosis of biliary epithelial cells: primary biliary cirrhosis and primary sclerosing cholangitis.

Primary biliary cirrhosis

Genetic Profiling of Colorectal Carcinomas of Patients with Primary Sclerosing Cholangitis and Inflammatory Bowel Disease

Natural History of Established and De Novo Inflammatory Bowel Disease After Liver Transplantation for Primary Sclerosing Cholangitis.

Overlap of IgG4-related Sclerosing Cholangitis and Primary Biliary Cirrhosis

Takayasu arteritis and primary sclerosing cholangitis: A casual association or different phenotypes of the same disease?

The immunobiology of primary sclerosing cholangitis

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Immunomodulatory Effect of Vancomycin on Treg in Pediatric Inflammatory Bowel Disease and Primary Sclerosing Cholangitis

Lymphocyte recruitment and homing to the liver in primary biliary cirrhosis and primary sclerosing cholangitis

Animal models in primary biliary cirrhosis and primary sclerosing cholangitis.

The immunophysiology and apoptosis of biliary epithelial cells: primary biliary cirrhosis and primary sclerosing cholangitis.

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Genetic Profiling of Colorectal Carcinomas of Patients with Primary Sclerosing Cholangitis and Inflammatory Bowel Disease

Natural History of Established and De Novo Inflammatory Bowel Disease After Liver Transplantation for Primary Sclerosing Cholangitis.

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Takayasu arteritis and primary sclerosing cholangitis: A casual association or different phenotypes of the same disease?

The immunobiology of primary sclerosing cholangitis