Primary biliary cholangitis. Part 1. State of the art, epidemiology, physiopathology and clinical manifestations

Primary biliary cholangitis (PBC) is a chronic autoimmune cholangiopathy characterized by a selective destruction of biliary epithelial cells of small and medium caliber hepatic ducts, which mainly affects women. The main symptoms are fatigue and pruritus, however, a large proportion of patients may...

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Published inRevista de gastroenterología del Perú Vol. 37; no. 4; pp. 357 - 364
Main Authors Rodríguez Lugo, Diego Andrés, Coronado Tovar, Jorge Julián, Solano Villamarin, Giovana Alejandra, Otero Regino, Wiliam
Format Journal Article
LanguageSpanish
Published Peru 01.10.2017
Subjects
Antibody Specificity
Autoantibodies - immunology
Autoimmune Diseases - epidemiology
Autoimmune Diseases - physiopathology
Bile Acids and Salts - metabolism
Cholangitis - complications
Cholangitis - epidemiology
Cholangitis - immunology
Cholangitis - physiopathology
Fatigue - etiology
Genetic Predisposition to Disease
Humans
Liver Cirrhosis, Biliary - etiology
Liver Cirrhosis, Biliary - prevention & control
Microbiota
Mitochondria - immunology
Pruritus - etiology
Smoking - adverse effects
Urinary Tract Infections - complications
Ursodeoxycholic Acid - therapeutic use
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Summary:Primary biliary cholangitis (PBC) is a chronic autoimmune cholangiopathy characterized by a selective destruction of biliary epithelial cells of small and medium caliber hepatic ducts, which mainly affects women. The main symptoms are fatigue and pruritus, however, a large proportion of patients may be asymptomatic. The diagnosis is based on AMA titers >1:40, alkaline phosphatase >1.5 times the upper limit for more than 24 weeks and compatible liver histology. It is associated with multiple autoimmune diseases mainly extrahepatic, thyroid diseases, bone diseases, among others. The first line treatment is ursodeoxycholic acid (UDCA), that improves liver function tests and delay the progression to cirrhosis. Currently, there are new treatments and adjuvant therapies on study. The purpose of this review is to offer an update in this topic, which is very important in gastroenterology and internal medicine. We formed an interdisciplinary team to search in the database Medline thorough PubMed with the key words describe below, we made a critical lecture of the titles and abstracts of each article to write this paper.
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ISSN:1609-722X