2022 Fiscal Year Final Research Report
Elucidation of the pathomechanisms of CIDP with anti-paranodal antibodies through the analysis of CNS and renal lesions
| Project/Area Number |
20K16602
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| Research Category |
Grant-in-Aid for Early-Career Scientists
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| Allocation Type | Multi-year Fund |
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| Review Section |
Basic Section 52020:Neurology-related
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| Research Institution | Kyushu University |
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Principal Investigator |
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| Project Period (FY) |
2020-04-01 – 2023-03-31
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| Keywords | neurofascin 155 / contactin-1 / 自己抗体 / IgG4 / 自己免疫性ノドパチー / 慢性炎症性脱髄性多発根ニューロパチー |
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| Outline of Final Research Achievements |
In the present study, we researched extra-peripheral nervous system involvement in autoimmune nodopathy, a new disease entity derived from chronic inflammatory demyelinating polyradiculoneuropathy. As a result, over half of the patients with IgG4 anti-neurofascin 155 antibody-positive nodopathy showed subclinical optic nerve abnormality on visual evoked potentials, while more than half of the patients with IgG4 anti-contactin-1 antibody-positive nodopathy showed overt proteinuria. It was also found that IgG4 anti-contactin-1 antibody-positive nodopathy was occasionally complicated by thymoma.All of them would be crucial to elucidate the pathophysiology of autoimmune nodopathy.
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| Free Research Field |
末梢神経学
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| Academic Significance and Societal Importance of the Research Achievements |
慢性炎症性脱髄性多発根ニューロパチーより派生した自己免疫性ノドパチーの末梢神経外の病変に注目し研究を行いました。その結果、IgG4抗neurofascin 155抗体陽性ノドパチー症例の視神経障害の程度、割合について明らかにしました。またIgG4抗contactin-1抗体陽性ノドパチー症例における、ネフローゼ症候群および顕性蛋白尿を呈する割合を明らかにしました。更に胸腺腫を合併する症例3例を見出しました。いずれも、新たな疾患概念である自己免疫性ノドパチーを特徴づける重要な所見です。
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