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 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.

• Fecha de publicación: 2017
• Institución: Universidad del Rosario
• Repositorio: Repositorio EdocUR - U. Rosario
• Idioma: eng
• 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
• Palabra clave: Alkaline phosphatase; Budesonide; Fibric acid derivative; Immunosuppressive agent; Mitochondrion antibody; Obeticholic acid; Ursodeoxycholic acid; Antiinflammatory agent; Budesonide; Chenodeoxycholic acid; Cholagogue; Immunosuppressive agent; Obeticholic acid; 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; Autoimmune disease; Biopsy; Cholangitis; Immunology; Liver; Pathology; Anti-inflammatory agents; Autoimmune diseases; Biopsy; Budesonide; Chenodeoxycholic acid; Cholagogues and choleretics; Cholangitis; Fibric acids; Humans; Immunosuppressive agents; Liver; Prognosis; Ursodeoxycholic acid; Antimitochondrial antibodies; Biliary epithelial cells; Epigenetics; Genetics; Obeticholic acid; Primary biliary cholangitis; Prognostic factors; Udca
• License: Abierto (Texto Completo)