Primary biliary cholangitis: Insights into genetic susceptibility and systemic manifestations
Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease characterized by damage to small intrahepatic bile ducts. The etiology and pathogenesis of PBC remain unknown. It is often considered to be related to an immunological disorder induced by genetics and environmental factors. Anti...
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| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
SAGE Publishing
2025-03-01
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| Series: | European Journal of Inflammation |
| Online Access: | https://doi.org/10.1177/1721727X251327293 |
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| Summary: | Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease characterized by damage to small intrahepatic bile ducts. The etiology and pathogenesis of PBC remain unknown. It is often considered to be related to an immunological disorder induced by genetics and environmental factors. Antimitochondrial antibodies (AMAs) along with specific antinuclear antibodies such as gp210 and Sp100 are specific markers of the disease. Middle-aged and elderly women are the main patients. The clinical manifestations of PBC are non-specific, which presents as fatigue and skin itching usually. However, patients gradually develop cholestasis and liver fibrosis, eventually dying as the disease progressed to cirrhosis and liver failure. Currently, ursodeoxycholic acid (UDCA) is the treatment of choice, which is recommended for all patients. PBC may coexist with other autoimmune disorders that may arise from multiple systems, such as inflammatory bowel disease (IBD), Sjögren’s syndrome (SS) and type 1 diabetes mellitus (T1DM). The causal relationship between PBC and extrahepatic autoimmune diseases (EHAIDs) is unclear. This article summarizes the new developments in the study of primary biliary cholangitis and aims to provide a reference for scientific and clinical workers in the field of research on this disease. |
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| ISSN: | 2058-7392 |