Current understanding of primary biliary cholangitis
Clinical and Molecular Hepatology
; : 1-21, 2021.
Article
em En
| WPRIM
| ID: wpr-874453
Biblioteca responsável:
WPRO
ABSTRACT
Primary biliary cholangitis (PBC) causes chronic and persistent cholestasis in the liver, eventually resulting in cirrhosis and hepatic failure without appropriate treatment. PBC mainly develops in middle-aged women, but it is also common in young women and men. PBC is considered a model of autoimmune disease because of the presence of diseasespecific autoantibodies, that is, antimitochondrial antibodies (AMAs), intense infiltration of mononuclear cells into the bile ducts, and a high prevalence of autoimmune diseases such as comorbidities. Histologically, PBC is characterized by degeneration and necrosis of intrahepatic biliary epithelial cells surrounded by a dense infiltration of mononuclear cells, coined as chronic non-suppurative destructive cholangitis, which leads to destructive changes and the disappearance of small- or medium-sized bile ducts. Since 1990, early diagnosis with the detection of AMAs and introduction of ursodeoxycholic acid as first-line treatment has greatly altered the clinical course of PBC, and liver transplantation-free survival of patients with PBC is now comparable to that of the general population.
Texto completo:
1
Base de dados:
WPRIM
Tipo de estudo:
Screening_studies
Idioma:
En
Revista:
Clinical and Molecular Hepatology
Ano de publicação:
2021
Tipo de documento:
Article