Apotopes and the biliary specificity of primary biliary cirrhosis

Apotopes and the biliary specificity of primary biliary cirrhosis.

UNLABELLED Primary biliary cirrhosis (PBC) is characterized by antimitochondrial antibodies (AMAs), directed to the E2 component of the pyruvate dehydrogenase complex (PDC-E2). Notwithstanding the presence of mitochondria in virtually all nucleated cells, the destruction in PBC is limited to small intrahepatic bile ducts. The reasons for this tissue specificity remain unknown, although biliary ...

Asymptomatic primary biliary cirrhosis.

Primary biliary cirrhosis.

Primary biliary cirrhosis is a chronic liver disease characterised by intrahepatic bile-duct destruction, cholestasis, and, in some cases, cirrhosis. Evidence supporting the autoimmune nature of this disorder includes the appearance of highly specific antimitochondrial antibodies (AMAs) and autoreactive T cells. Concordance rates in monozygotic twins, familial prevalence, and genetic associatio...

Primary Biliary Cirrhosis

Primary Biliary Cirrhosis (PBC) is the result of long-standing damage to intra-hepatic biliary channels as a result of immune mechanisms leading to cholestasis and its associated complications. It can end up in chronic liver disease or cirrhosis. It usually manifests as pruritis that then proceeds to fatigue, jaundice and other features of chronic liver disease. It is detected by raised serolog...

Primary biliary cirrhosis.

Primary biliary cirrhosis (PBC) is a chronic, cholestatic liver disease that is characterized by progressive immune mediated destruction of the intrahepatic bile ducts. Over time, fibrosis extends out of the portal tracts and progresses to cirrhosis. Neither the etiology nor the pathogenesis are well understood; however, most of the current evidence suggests that it is an autoimmune condition t...