| Project/Area Number |
17591060
|
|---|
| Research Category |
Grant-in-Aid for Scientific Research (C)
|
| Allocation Type | Single-year Grants |
|---|
| Section | 一般 |
|---|
| Research Field |
膠原病・アレルギー・感染症内科学
|
|---|
| Research Institution | KANAZAWAMEDICALUNIVERSITY |
|---|
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
|
| Project Status |
Completed (Fiscal Year 2007)
|
| Budget Amount *help |
¥3,410,000 (Direct Cost: ¥3,200,000、Indirect Cost: ¥210,000)
Fiscal Year 2007: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2006: ¥1,200,000 (Direct Cost: ¥1,200,000)
Fiscal Year 2005: ¥1,300,000 (Direct Cost: ¥1,300,000)
|
|---|
| Keywords | Mikulicz's diseae / Siogren's syndrome / Autoimmune pancreatitis / IgG Subclass / glucocorticoids / リンパ増殖性疾患 / AMOLPS / Mikulicz病 / Sjogren症候群 / 間質性肺炎 / 間質性腎炎 |
|---|
| 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.
|
