title: Proposed therapies in primary biliary cholangitis.
abstract: Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is a model autoimmune disease with chronic cholestasis characterized by the hallmark of anti-mitochondrial antibodies and treated with ursodeoxycholic acid (UDCA). However, approximately 20-40% of patients incompletely respond to UDCA and have an increased risk of disease progression. Although there have been significant advances in the immunobiology of PBC, these have yet to be translated into newer therapeutic modalities. Current approaches to controlling the immune response include broad immunosuppression with corticosteroids as well as targeted therapies directed against T and B cells. In contrast, ameliorating cholestasis is the focus of other therapies in development, including obeticholic acid. In this article the authors will discuss ongoing clinical trials and, in particular, the rationale for choosing agents that may effectively target the aberrant immune response.
authors.affiliation: Department of Surgery, Oncology and Gastroenterology, University of Padova, Italy.
authors.forename: Annarosa
authors.initials: A
authors.lastname: Floreani
authors.affiliation: Division of Rheumatology, Allergy, and Clinical Immunology, University of California Davis School of Medicine, Davis, CA, USA.|Diagnostic and Treatment Center for Non-Infectious Liver Diseases, 302 Military Hospital, Beijing, China.
authors.forename: Ying
authors.initials: Y
authors.lastname: Sun
authors.affiliation: Diagnostic and Treatment Center for Non-Infectious Liver Diseases, 302 Military Hospital, Beijing, China.
authors.forename: Zheng Sheng
authors.initials: ZS
authors.lastname: Zou
authors.affiliation: Diagnostic and Treatment Center for Non-Infectious Liver Diseases, 302 Military Hospital, Beijing, China.
authors.forename: Baosen
authors.initials: B
authors.lastname: Li
authors.affiliation: Department of Surgery, Oncology and Gastroenterology, University of Padova, Italy.
authors.forename: Nora
authors.initials: N
authors.lastname: Cazzagon
authors.affiliation: Division of Gastroenterology and Hepatology, University of California Davis, Davis, CA, USA.
authors.forename: Christopher L
authors.initials: CL
authors.lastname: Bowlus
authors.affiliation: Division of Rheumatology, Allergy, and Clinical Immunology, University of California Davis School of Medicine, Davis, CA, USA.
authors.forename: M Eric
authors.initials: ME
authors.lastname: Gershwin
mesh_terms: D000818:Animals; D001647:Bile Acids and Salts / Q000378:metabolism; D001659:Biliary Tract / Q000187:drug effects* / Q000276:immunology / Q000378:metabolism; D001688:Biological Products / Q000627:therapeutic use; D002756:Cholagogues and Choleretics / Q000009:adverse effects / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D008105:Liver Cirrhosis, Biliary / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology / Q000378:metabolism; D033581:Stem Cell Transplantation; D016896:Treatment Outcome
chemical_list: D001647:Bile Acids and Salts; D001688:Biological Products; D002756:Cholagogues and Choleretics; D007166:Immunosuppressive Agents
keywords: Primary biliary cirrhosis; UDCA; biologic agents; budesonide; fatigue; fibrates; obeticholic acid; pruritus; treatment