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Imaging of autoimmune biliary disease

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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.

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Authors and Affiliations

  1. Department of Radiology and Biomedical Imaging, University of California San Francisco, 505 Parnassus Ave, San Francisco, CA, 94143-0628, USA

    Melinda J. Yeh, Spencer C. Behr & Benjamin M. Yeh

  2. 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

    So Yeon Kim

  3. 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

    Kartik S. Jhaveri

  4. Department of Radiology, Research Institute of Radiological Science, Severance Hospital, Yonsei University College of Medicine, Seoul, 03722, Korea

    Nieun Seo

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  1. Melinda J. Yeh
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  2. So Yeon Kim
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  3. Kartik S. Jhaveri
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  4. Spencer C. Behr
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  6. Benjamin M. Yeh
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Correspondence to Benjamin M. Yeh.

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Conflict of interest

Melinda Yeh declares that she has no conflict of interest. So Yeon Kim declares that she has no conflict of interest. Kartik Jhaveri declares that he has no conflict of interest. Spencer Behr has received research grants from and is a consultant for General Electric Healthcare. Nieun Seo declares that she has no conflict of interest. Benjamin Yeh has received research grants from the National Institutes of Health and General Electric Healthcare, book royalties from Oxford University Press, and is a shareholder in Nextrast, inc.

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Yeh, M.J., Kim, S.Y., Jhaveri, K.S. et al. Imaging of autoimmune biliary disease. Abdom Radiol 42, 3–18 (2017). https://doi.org/10.1007/s00261-016-0903-8

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