• Internal medicine · Jan 2013

    Case Reports

    Two siblings with type 1 autoimmune pancreatitis.

    • Takayuki Watanabe, Masahiro Maruyama, Tetsuya Ito, Keita Kanai, Takaya Oguchi, Takashi Muraki, Hideaki Hamano, Norikazu Arakura, Masao Ota, and Shigeyuki Kawa.
    • Department of Gastroenterology, Shinshu University School of Medicine, Japan.
    • Intern. Med. 2013 Jan 1; 52 (8): 895-9.

    AbstractType 1 autoimmune pancreatitis (AIP) is characterized by a high serum IgG4 concentration and is closely associated with the HLA-DRB1(*)04:05-DQB1(*)04:01 haplotype, for which family studies may disclose its immunogenetic significance. In the present study, we encountered two male siblings with type 1 AIP who exhibited diffuse pancreatic swelling with a capsule-like rim and diffuse pancreatic duct stricture. The younger brother also displayed characteristic IgG4-related sialadenitis and retroperitoneal fibrosis. Contrary to our expectations, the siblings showed only normal or slightly elevated values of serum IgG4 and no HLA DRB1(*)04:05-DQB1(*)04:01 haplotype, suggesting that type 1 autoimmune pancreatitis is associated with multiple immunogenetic factors.

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