Abstract
Primary biliary cirrhosis (PBC) is a chronic progressive autoimmune cholestatic liver disease characterized by highly specific antimitochondrial antibodies (AMAs) and the specific immune-mediated injury of small intrahepatic bile ducts. Unique apoptotic feature of biliary epithelial cells (BECs) may contribute to apotope presentation to the immune system, causing unique tissue damage in PBC. Perpetuation of inflammation may result in senescence of BECs, contributing to irreversible loss of bile duct. In addition to the classic liver manifestations, focal inflammation and tissue damage are also seen in salivary glands and urinary tract in a significant proportion of PBC patients. These findings provide potent support to the idea that molecular mimicry may be involved in the breakdown of autoimmune tolerance and mucosal immunity may lead to a systematic epithelitis in PBC patients. Thus, PBC is considered a generalized epithelitis in clinical practice.
Highlights
Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease characterized by an immune-mediated inflammatory destruction of small intrahepatic bile ducts
PBC were hypersensitive to LPS, and LPS could induce biliary epithelial cells (BECs) to secret inflammatory factors or chemokines such as IL-6, MCP-1 and IL-8. These results have demonstrated that BECs from PBC
Defect in the clearance of post apoptosis protein in the apotope and leakage of intact autoantigen to the immune system leading to constant inflammatory response may be mechanistically responsible for bile duct injury
Summary
Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease characterized by an immune-mediated inflammatory destruction of small intrahepatic bile ducts. The natural history of PBC varies considerably across all patients, ranging from an asymptomatic and stable condition with an insidious and slow progression, to a troublesome disorder of repetitious exacerbations and remissions, to an even severe disease of highly symptomatic and rapidly fatal. Female patients with PBC are more prone to develop recurrent UTI compared to the patients with other chronic liver diseases [6]. It has been confirmed that PBC can exhibit a progressive immune-mediated epithelial destruction of the biliary tract, salivary gland and urinary tract. Gershwin has suggested that the term “autoimmune epithelitis” would accurately reflect the condition for the immune-mediated injury of the target epithelial elements in PBC and SS [9]. Biliary Epithelial Cells (BECs) Play a Dominant Role in the Development of PBC
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