2022 Fiscal Year Final Research Report
Elucidation of autoimmune mechanisms in airway mucosal damage in Stevens-Johnson syndrome
Project/Area Number |
20K17188
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Research Category |
Grant-in-Aid for Early-Career Scientists
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Allocation Type | Multi-year Fund |
Review Section |
Basic Section 53030:Respiratory medicine-related
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Research Institution | Kyoto Prefectural University of Medicine |
Principal Investigator |
Kaneko Yoshiko 京都府立医科大学, 医学(系)研究科(研究院), 講師 (30768825)
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Project Period (FY) |
2020-04-01 – 2023-03-31
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Keywords | Stevens-Johnson症候群 / 気道粘膜障害 / 閉塞性細気管支炎 / 自己抗体 |
Outline of Final Research Achievements |
In this study, we investigated commonalities and differences with paraneoplastic syndrome PNP, whose clinical features are very similar, in patients with chronic SJS/TEN suggesting the presence of airway mucosal disorders. The purpose of this study was to explore autoimmune mechanisms involved in the pathogenesis of inflammation. Autoantibodies against periplakin protein, one of the plakin family proteins characteristic of PNP, were significantly detected in SJS/TEN patients with chronic respiratory disease. It was suggested that it may be a factor predicting the presence of peripheral bronchial mucosal/alveolar damage in SJS/TEN.
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Free Research Field |
呼吸器内科
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Academic Significance and Societal Importance of the Research Achievements |
Stevens-Johnson症候群 (SJS) およびその重症型である中毒性表皮壊死融解症 (TEN)はNSAIDSや感冒薬・抗てんかん薬内服を契機に発症する重症薬疹である。急性期は体幹・四肢に水疱・びらんを主体とした多彩な皮膚病変や失明に至る重篤な眼合併症を認める。腸粘膜障害や尿管粘膜など全身の粘膜に障害を起こすほか、呼吸器合併症としては閉塞性細気管支炎が報告されるが、頻度も少なくその病態もあきらかではない。BO併発症例は呼吸不全による死亡率が高く、気道粘膜炎症の病態解明は、患者に適切かつ有効な治療を提供するためにも切に望まれている。
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