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2007 Fiscal Year Final Research Report Summary

Autoimwune multi-tagemlympliopmliferative syndrome (AMOLPS)-propoeal for a new clinical entily

Research Project

Project/Area Number 17591060
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field 膠原病・アレルギー・感染症内科学
Research InstitutionKANAZAWAMEDICALUNIVERSITY

Principal Investigator

MASAKI Yasufumi  KANAZAWAMEDICALUNIVERSITY, School of Medicine, Associate Professor (40238895)

Co-Investigator(Kenkyū-buntansha) SUGAI Susumu  KANAZAWAMEDICALUNIVERSITY, School of Medicine, Emeritus Professor (20064537)
UMEHARA Hisanori  KANAZAWAMEDICALUNIVERSITY, School of Medicine, Professor PROFESSOR (70247881)
TAKAHAAHI Haroki  Sapporo Medical University, School of Medicine, Senior Assistant, Professor (00264523)
KITAGAWA Kazuko  KANAZAWAMEDICALUNIVERSITY, School of Medicine, Professor PROFESSOR (60102042)
NISHIMORI Isao  Kochi Metrical School, Medicine, Senior Assistant Professor (30237747)
Project Period (FY) 2005 – 2007
KeywordsMikulicz's diseae / Siogren's syndrome / Autoimmune pancreatitis / IgG Subclass / glucocorticoids
Research Abstract

We investigated patients with MD and IgG4-related disorders registered from all over Japan, and set up provisional criteris for the new clinical entity : IgG4-positive multi-organ lymphoproliferative syndrome (IgG4-AMOLPS). The diagnostic preliminary criteria include elevated serum IgG4 (>135mg/dl) and infiltrution of IgG4- plasma cells in the tissue (IgG4'/IgG^+ plasma cells >50%) with fibrosis or selerosis. We compared 64 patients with 1gG4-AMOLPS and 31 patients with typical SS in clinical features. laboratory data and pathologies.
Results : The incidences of xeroatomia, xerophtbalmia and arthralgia, rheumatoid factor and anti-nuclear, anti-SSA/Ro and anti-SSE/La antibodies were significantly lower in IgG4-AMOLPS than typical SS patients. Allergic rhinitis and autoimmune pancreatitis were significantly more frequent and total IgG. IgG2, IgG4 and IgE were significantly elevated in IgG4'AMOLPS. Histological specimen from patients with IgG4-AMOLPS revealed marked IgG4- plasma cell infiltration. Many patients with IgG4-AMOLPS showed lymphocyte follicle formation, but lymphoepithelial lesions were rare. Few IgG4' cells were seen in the tissue of typical SS. Thirty-eight patients with IgG4-AMOLPS treated with glucocorticoids showed marked clinical improvements.
Conclusion : Despite similarities in the involved organs, there are marked clinical and pathological differences between IgG4^+AMOLPS and SS. Based on the clinical features and good response to giucocorticoid, we propose a new clinical entity : IgG4^+AMOLPS.

  • Research Products

    (10 results)

All 2008 2007 Other

All Journal Article (6 results) (of which Peer Reviewed: 1 results) Presentation (2 results) Remarks (2 results)

  • [Journal Article] 自己免疫性膵炎の新展開2008

    • Author(s)
      西森功、
    • Journal Title

      日本消化器病学会雑誌 105(4)

      Pages: 511-528

    • Description
      「研究成果報告書概要(和文)」より
  • [Journal Article] The new development of autoimmune pancreatitis2008

    • Author(s)
      Nishimori L.
    • Journal Title

      Japanese J Gastorocnterol 105-4

      Pages: 511-528

    • Description
      「研究成果報告書概要(欧文)」より
  • [Journal Article] IgG4とリウマチ性疾患2007

    • Author(s)
      正木康史、
    • Journal Title

      リウマチ科 38(4)

      Pages: 404-408

    • Description
      「研究成果報告書概要(和文)」より
  • [Journal Article] Clonality analysis of lymphoproliferative disorders in patients with Sjogren's syodrome2007

    • Author(s)
      Dong L
    • Journal Title

      Clin Exp Immunol. 150(2)

      Pages: 279-84

    • Description
      「研究成果報告書概要(和文)」より
    • Peer Reviewed
  • [Journal Article] IgG4 and rheumatic diseuses2007

    • Author(s)
      Masaki Y.
    • Journal Title

      Rheumatology 38-4

      Pages: 404-408

    • Description
      「研究成果報告書概要(欧文)」より
  • [Journal Article] Clonelity analysis of lymphoproliferative disordere in pationts with Sjogren's syndrome2007

    • Author(s)
      Dong L.
    • Journal Title

      Clin Exp Immunol 150-2

      Pages: 279-284

    • Description
      「研究成果報告書概要(欧文)」より
  • [Presentation] 64例のIgG4+AMOLPSの臨床的検討-Sjogren症候群との差異2007

    • Author(s)
      近木康史
    • Organizer
      日本シェーグレン症候群研究会
    • Place of Presentation
      京都
    • Year and Date
      2007-09-21
    • Description
      「研究成果報告書概要(和文)」より
  • [Presentation] Retrospcctive analysis of 64 cases of IgG4+ AMOLPS, the difference with Sjogren's syndromc2007

    • Author(s)
      Masuki Y.
    • Organizer
      The 16st Japanese medical soeiety of the Sjogren'e syndrome
    • Place of Presentation
      Kyoto, Japan
    • Year and Date
      2007-09-21
    • Description
      「研究成果報告書概要(欧文)」より
  • [Remarks] 「研究成果報告書概要(和文)」より

    • URL

      http://www.kanazawa-med.ac.jp/%7Ehematol/MD.html

  • [Remarks] 「研究成果報告書概要(和文)」より

    • URL

      http://www.kanazawa-med.ac.jp/~hematol/MD.html

URL: 

Published: 2011-06-18  

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