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Primary biliary cholangitis: a comprehensive overview
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Autores
Lleo A.
Marzorati S.
Anaya, Juan-Manuel
Gershwin M.E.
Fecha
2017
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Springer
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Abstract
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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Keywords
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
