2007 Fiscal Year Final Research Report Summary
Autoimwune multi-tagemlympliopmliferative syndrome (AMOLPS)-propoeal for a new clinical entily
| Project/Area Number |
17591060
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
膠原病・アレルギー・感染症内科学
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| Research Institution | KANAZAWAMEDICALUNIVERSITY |
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Principal Investigator |
MASAKI Yasufumi KANAZAWAMEDICALUNIVERSITY, School of Medicine, Associate Professor (40238895)
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| 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)
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| Project Period (FY) |
2005 – 2007
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| Keywords | Mikulicz's diseae / Siogren's syndrome / Autoimmune pancreatitis / IgG Subclass / glucocorticoids |
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| 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.
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Research Products
(10 results)
