title: Imaging of autoimmune biliary disease.
abstract: Autoimmune biliary diseases are poorly understood but important to recognize. Initially, autoimmune biliary diseases are asymptomatic but may lead to progressive cholestasis with associated ductopenia, portal hypertension, cirrhosis, and eventually liver failure. The three main forms of autoimmune biliary disease are primary biliary cirrhosis, primary sclerosing cholangitis, and IgG4-associated cholangitis. Although some overlap may occur between the three main autoimmune diseases of the bile ducts, each disease typically affects a distinct demographic group and requires a disease-specific diagnostic workup. For all the autoimmune biliary diseases, imaging provides a means to monitor disease progression, assess for complications, and screen for the development of hepatobiliary malignancies that are known to affect patients with these diseases. Imaging is also useful to suggest or corroborate the diagnosis of primary sclerosing cholangitis and IgG4-associated cholangitis. We review the current literature and emphasize radiological findings and considerations for these autoimmune diseases of the bile ducts.
authors.affiliation: Department of Radiology and Biomedical Imaging, University of California San Francisco, 505 Parnassus Ave, San Francisco, CA, 94143-0628, USA.
authors.forename: Melinda J
authors.initials: MJ
authors.lastname: Yeh
authors.affiliation: Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, 388-1, Pungnap 2-dong, Songpa-ku, Seoul, 138-736, Korea.
authors.forename: So Yeon
authors.initials: SY
authors.lastname: Kim
authors.affiliation: Division of Abdominal Imaging, Joint Department of Medical Imaging, University Health Network, Mt. Sinai Hospital and Women's College Hospital, University of Toronto, 610 University Ave, Toronto, ON, M5G 2M9, Canada.
authors.forename: Kartik S
authors.initials: KS
authors.lastname: Jhaveri
authors.affiliation: Department of Radiology and Biomedical Imaging, University of California San Francisco, 505 Parnassus Ave, San Francisco, CA, 94143-0628, USA.
authors.forename: Spencer C
authors.initials: SC
authors.lastname: Behr
authors.affiliation: Department of Radiology, Research Institute of Radiological Science, Severance Hospital, Yonsei University College of Medicine, Seoul, 03722, Korea.
authors.forename: Nieun
authors.initials: N
authors.lastname: Seo
authors.affiliation: Department of Radiology and Biomedical Imaging, University of California San Francisco, 505 Parnassus Ave, San Francisco, CA, 94143-0628, USA. Ben.Yeh@ucsf.edu.
authors.forename: Benjamin M
authors.identifier: 0000-0003-2174-3009
authors.initials: BM
authors.lastname: Yeh
mesh_terms: D001327:Autoimmune Diseases / Q000150:complications / Q000000981:diagnostic imaging*; D001660:Biliary Tract Diseases / Q000150:complications / Q000000981:diagnostic imaging*; D003937:Diagnosis, Differential; D018450:Disease Progression; D006801:Humans
keywords: Autoimmune biliary disease; Bile duct; Hepatobiliary; Primary biliary cirrhosis; Sclerosing cholangitis