Primary biliary cholangitis: a comprehensive overview
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by biliary destruction, progressive cholestasis, and potentially liver cirrhosis. Patients develop a well-orchestrated immune reaction, both innate and adaptive, against mitochondrial antigens that specifically targets in...
| Autores: | , , , |
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| Tipo de recurso: | artículo |
| Estado: | Versión publicada |
| Fecha de publicación: | 2017 |
| País: | Colombia |
| Institución: | Universidad del Rosario |
| Repositorio: | Repositorio EdocUR - U. Rosario |
| Idioma: | inglés |
| OAI Identifier: | oai:repository.urosario.edu.co:10336/24193 |
| Acceso en línea: | https://doi.org/10.1007/s12072-017-9830-1 https://repository.urosario.edu.co/handle/10336/24193 |
| Access Level: | acceso abierto |
| Palabra clave: | Alkaline phosphatase Budesonide Fibric acid derivative Immunosuppressive agent Mitochondrion antibody Obeticholic acid Ursodeoxycholic acid Antiinflammatory agent Chenodeoxycholic acid Cholagogue Adaptive immunity Autoimmune disease Clinical feature Disease course End stage liver disease Histopathology Human Immunofluorescence Innate immunity Liver biopsy Liver transplantation Nonhuman Pathogenesis Pathophysiology Patient care Primary biliary cirrhosis Priority journal Prognosis Review Risk factor Treatment response Analogs and derivatives Biopsy Cholangitis Immunology Liver Pathology Anti-inflammatory agents Autoimmune diseases Cholagogues and choleretics Fibric acids Humans Immunosuppressive agents Antimitochondrial antibodies Biliary epithelial cells Epigenetics Genetics Primary biliary cholangitis Prognostic factors Udca |
| Sumario: | Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by biliary destruction, progressive cholestasis, and potentially liver cirrhosis. Patients develop a well-orchestrated immune reaction, both innate and adaptive, against mitochondrial antigens that specifically targets intrahepatic biliary cells. A puzzling feature of PBC is that the immune attack is predominantly organ specific, although the mitochondrial autoantigens are found in all nucleated cells. The disease results from a combination of genetic and environmental risk factors; however, the exact pathogenesis remains unclear. Serologically, PBC is characterized by presence of antimitochondrial antibodies, which are present in 90–95 % of patients and are often detectable years before clinical signs appear. Like other complex disorders, PBC is heterogeneous in its presentation, symptomatology, disease progression, and response to therapy. A significant number of patients develop end-stage liver disease and eventually require liver transplantation. Recent studies from large international cohorts have better identified prognostic factors, suggesting a change in patient management based on risk stratification. Therapeutic options are changing. In this review we discuss data on the autoimmune responses and treatment of the disease. © 2017, Asian Pacific Association for the Study of the Liver. |
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