Primary biliary cirrhosis and autoimmune cholangitis

Carlo Selmi, Ana Lleo, Pietro Invernizzi, M. Eric Gershwin

Research output: Chapter in Book/Report/Conference proceedingChapter

1 Citation (Scopus)

Abstract

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease of unknown etiology characterized by hightiter serum antimitochondrial autoantibodies (AMAs) and an autoimmune-mediated destruction of the small and mediumsized intrahepatic bile ducts. From a clinical standpoint, PBC is a peculiar, yet representative, autoimmune disease (Table). It affects women more frequently than men, with a female-to-male ratio of 9 to 1, and the average age at diagnosis is within the fifth and sixth decades of life, with exceptional cases described in pediatric ages. Epidemiological data indicate a geographical pattern of PBC prevalence and incidence rates, which are higher in northern countries (England, Scandinavia, northern United States). The diagnosis of PBC is made when two of three criteria are fulfilled, i.e., presence of serum AMAs, increased enzymes indicating cholestasis (i.e., alkaline phosphatase) for longer than 6 mo, and a compatible or diagnostic liver histology. Clinical symptoms include fatigue, pruritus, and jaundice. The progression of PBC varies widely for unknown reasons, as represented by certain patients remaining asymptomatic and others reaching liver failure at young ages.

Original languageEnglish (US)
Title of host publicationLiver Immunology: Principles and Practice
PublisherHumana Press
Pages235-247
Number of pages13
ISBN (Print)9781588298188
DOIs
StatePublished - 2007

Fingerprint

Cholangitis
Biliary Liver Cirrhosis
Autoantibodies
Scandinavian and Nordic Countries
Intrahepatic Bile Ducts
Cholestasis
Liver Failure
Pruritus
Jaundice
Serum
England
Autoimmune Diseases
Fatigue
Alkaline Phosphatase
Liver Diseases
Histology
Pediatrics
Liver
Incidence
Enzymes

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Selmi, C., Lleo, A., Invernizzi, P., & Gershwin, M. E. (2007). Primary biliary cirrhosis and autoimmune cholangitis. In Liver Immunology: Principles and Practice (pp. 235-247). Humana Press. https://doi.org/10.1007/978-1-59745-518-3_19

Primary biliary cirrhosis and autoimmune cholangitis. / Selmi, Carlo; Lleo, Ana; Invernizzi, Pietro; Gershwin, M. Eric.

Liver Immunology: Principles and Practice. Humana Press, 2007. p. 235-247.

Research output: Chapter in Book/Report/Conference proceedingChapter

Selmi, C, Lleo, A, Invernizzi, P & Gershwin, ME 2007, Primary biliary cirrhosis and autoimmune cholangitis. in Liver Immunology: Principles and Practice. Humana Press, pp. 235-247. https://doi.org/10.1007/978-1-59745-518-3_19
Selmi C, Lleo A, Invernizzi P, Gershwin ME. Primary biliary cirrhosis and autoimmune cholangitis. In Liver Immunology: Principles and Practice. Humana Press. 2007. p. 235-247 https://doi.org/10.1007/978-1-59745-518-3_19
Selmi, Carlo ; Lleo, Ana ; Invernizzi, Pietro ; Gershwin, M. Eric. / Primary biliary cirrhosis and autoimmune cholangitis. Liver Immunology: Principles and Practice. Humana Press, 2007. pp. 235-247
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