Two Siblings with Type 1 Autoimmune Pancreatitis

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  • Watanabe Takayuki
    Department of Gastroenterology, Shinshu University School of Medicine, 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
  • Kawa Shigeyuki
    Center for Health, Safety and Environment Management, Shinshu University, Japan

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Description

Type 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.<br>

Journal

  • Internal Medicine

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

    The Japanese Society of Internal Medicine

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