Abstract
Autoimmune pancreatitis, the pancreatic manifestation of IgG4-RD, is frequently complicated by bile duct lesions that extend mainly from the papilla to bile ducts and gallbladder. The differentiation of these bile duct lesions from primary sclerosing cholangitis and bile duct cancer can be difficult in some cases, but this distinction is critical because the therapeutic approaches differ markedly.
In IgG4-related sclerosing cholangitis, as in other forms of IgG4-RD, glucocorticoid therapy is usually highly effective, but disease relapses are encountered frequently. Because bile ducts are comprised of inherently narrow lumens, disease recurrence can lead directly to ductal stenosis. This concern, unfortunately, necessitates the long-term use of maintenance glucocorticoids in some patients, with all of the attendant morbidity associated with this treatment.
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Muraki, T., Hamano, H., Kawa, S. (2014). Bile Duct Lesions. In: Umehara, H., Okazaki, K., Stone, J., Kawa, S., Kawano, M. (eds) IgG4-Related Disease. Springer, Tokyo. https://doi.org/10.1007/978-4-431-54228-5_11
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DOI: https://doi.org/10.1007/978-4-431-54228-5_11
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