Hôpital Saint-Antoine, service d’hépatologie, centre de référence des maladies inflammatoires des voies biliaires, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France
INSERM UMR_S 938, CDR Saint-Antoine, Université Pierre et Marie Curie, Paris
- Key words: cholestasis, primary biliary cholangitis, primary sclerosing cholangitis, IgG4 related hepatobiliary disease, ursodeoxycholic acid
- DOI : 10.1684/hpg.2017.1518
- Page(s) : 857-65
- Published in: 2017
Autoimmune cholangitis are uncommon chronic cholestatic diseases including distinct medical conditions: primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and IgG4 related hepatobiliary disease. Their common pathophysiology includes an excessive activation of immune response leading to inflammatory injuries of biliary ducts at differents levels exposing to specific complications. Autoimmune cholangitis share common clinical and biological caracteristics: pruritus and chronic cholestasis but they occur in different settings (defined by sex, age and associated diseases). Diagnosis is based on results of specific biological tests (antimitochondrial antibodies and serum IgG4 levels), biliary tract imaging and liver histology. Treatment of PBC and PSC is mainly ursodeoxycholic acid and liver transplantation but clinical research is very active to identify new treatments for both symptoms and cause of these diseases. IgG4 related hepatobiliary disease displays a remarkable response to corticotherapy which is used as a diagnostic test. This mini-review aims to deliver didactic messages about current management of autoimmune cholangitis.