Two Siblings with Type 1 Autoimmune Pancreatitis

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Author(s)

    • Watanabe Takayuki
    • Department of Gastroenterology, Shinshu University School of Medicine, Japan
    • Kawa Shigeyuki
    • Center for Health, Safety and Environment Management, Shinshu University, Japan
    • Maruyama Masahiro
    • Department of Gastroenterology, Shinshu University School of Medicine, Japan
    • Ito Tetsuya
    • Department of Gastroenterology, Shinshu University School of Medicine, Japan
    • Kanai Keita
    • Department of Gastroenterology, Shinshu University School of Medicine, Japan
    • Oguchi Takaya
    • Department of Gastroenterology, Shinshu University School of Medicine, Japan
    • Muraki Takashi
    • Department of Gastroenterology, Shinshu University School of Medicine, Japan
    • Hamano Hideaki
    • Department of Gastroenterology, Shinshu University School of Medicine, Japan
    • Arakura Norikazu
    • Department of Endoscopic Examination Center, Shinshu University School of Medicine, Japan
    • Ota Masao
    • Department of Legal Medicine, Shinshu University School of Medicine, Japan

Abstract

Type 1 autoimmune pancreatitis (AIP) is characterized by a high serum IgG4 concentration and is closely associated with the <i>HLA-DRB1<sup>*</sup>04:05-DQB1<sup>*</sup>04:01</i> 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 <i>HLA DRB1<sup>*</sup>04:05-DQB1<sup>*</sup>04:01</i> haplotype, suggesting that type 1 autoimmune pancreatitis is associated with multiple immunogenetic factors.<br>

Journal

  • Internal Medicine

    Internal Medicine 52(8), 895-899, 2013

    The Japanese Society of Internal Medicine

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