[Primary biliary cirrhosis]. 2003

Silvija Cuković-Cavka
Zavod za gastroenterologiju, Klinicki bolnicki centar Zagreb, Kispatićeva 12, 10000 Zagreb, Hrvatska.

Primary biliary cirrhosis is an autoimmune chronic cholestatic liver disease of unknown cause that usually affects middle-aged women. It is characterized by inflammatory destruction of the interlobular and septal bile ducts, which leads to chronic cholestasis and cirrhosis. The diagnosis should be considered in the setting of elevated alkaline phosphatase, immunoglobulin M level and the presence of antimitochondrial antibody in serum. Ursodeoxycholic acid is the only medication of proven benefit for these patients. Liver transplantation is only therapeutic option for patients who have end-stage disease.

UI MeSH Term Description Entries
D008105 Liver Cirrhosis, Biliary FIBROSIS of the hepatic parenchyma due to obstruction of BILE flow (CHOLESTASIS) in the intrahepatic or extrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC; BILE DUCTS, EXTRAHEPATIC). Primary biliary cholangitis involves the destruction of small intra-hepatic bile ducts and decreased bile secretion. Secondary biliary cholangitis is produced by prolonged obstruction of large intrahepatic or extrahepatic bile ducts from a variety of causes. Biliary Cirrhosis,Biliary Cirrhosis, Primary,Biliary Cirrhosis, Secondary,Cholangitis, Chronic Nonsuppurative Destructive,Liver Cirrhosis, Obstructive,Primary Biliary Cholangitis,Biliary Cirrhosis, Primary, 1,Primary Biliary Cirrhosis,Secondary Biliary Cholangitis,Secondary Biliary Cirrhosis,Biliary Cholangitides, Primary,Biliary Cholangitis, Primary,Biliary Cholangitis, Secondary,Cholangitides, Primary Biliary,Cholangitis, Primary Biliary,Cholangitis, Secondary Biliary,Cirrhosis, Biliary,Cirrhosis, Secondary Biliary,Liver Cirrhoses, Biliary,Obstructive Liver Cirrhosis,Primary Biliary Cholangitides,Secondary Biliary Cholangitides
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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