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Intern Med. 2018 Jun 15;57(12):1725-1732. doi: 10.2169/internalmedicine.9818-17. Epub 2017 Dec 21.

Autoimmune Hemolytic Anemia Obscured by the Obstructive Jaundice Associated with IgG4-related Sclerosing Cholangitis in a Patient with Type 1 Autoimmune Pancreatitis: A Case Report and Review of the Literature.

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Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Japan.
Department of Hematology and Oncology, Nagoya City University Graduate School of Medical Sciences, Japan.


Type 1 autoimmune pancreatitis (AIP) is a pancreatic manifestation of IgG4-retated disease that is often associated with IgG4-related sclerosing cholangitis (IgG4-SC). Autoimmune hemolytic anemia (AIHA) is an immune-related disease that causes hemolytic anemia. Although type 1 AIP/IgG4-SC and AIHA have a shared etiology as a presumed autoimmune disease, they rarely overlap, and their association has not been clarified. Secondary AIHA might not be diagnosed appropriately because the obstructive jaundice observed in type 1 AIP/IgG4-SC can obscure the presence of hemolytic jaundice. We herein report a case of type 1 AIP/IgG4-SC overlapping with secondary AIHA along with a review of the literature.


IgG4-related disease (IgG4-RD); IgG4-related sclerosing cholangitis (IgG4-SC); autoimmune hemolytic anemia (AIHA); autoimmune pancreatitis (AIP)

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