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Elucidation of autoimmune mechanisms in airway mucosal damage in Stevens-Johnson syndrome

Research Project

Project/Area Number 20K17188
Research Category

Grant-in-Aid for Early-Career Scientists

Allocation TypeMulti-year Fund
Review Section Basic Section 53030:Respiratory medicine-related
Research InstitutionKyoto 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)

Project Period (FY) 2020-04-01 – 2023-03-31
Project Status Completed (Fiscal Year 2022)
Budget Amount *help
¥3,510,000 (Direct Cost: ¥2,700,000、Indirect Cost: ¥810,000)
Fiscal Year 2022: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
Fiscal Year 2021: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2020: ¥390,000 (Direct Cost: ¥300,000、Indirect Cost: ¥90,000)
KeywordsStevens-Johnson症候群 / 気道粘膜障害 / 閉塞性細気管支炎 / 自己抗体 / 腫瘍随伴天疱瘡 / プラキンファミリー蛋白抗体 / 腫瘍随伴性天疱瘡 / 抗エピプラキン抗体
Outline of Research at the Start

SSJS/TENとPNPは、全身皮膚粘膜病変と閉塞性細気管支炎という非常に酷似した臨床像を持ち、共通する病態機構があることが示唆される。、本研究では、PNP発症に関連する自己抗体についてSJS/TENの免疫血清学的検討を行い発症機序に関わる因子を検討する。両疾患に共通する自己免疫的要因が同定されれば、SJS/TENの病態解明に大きく寄与する可能性がある。

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.

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。

Report

(4 results)
  • 2022 Annual Research Report   Final Research Report ( PDF )
  • 2021 Research-status Report
  • 2020 Research-status Report
  • Research Products

    (4 results)

All 2021 2020

All Journal Article (1 results) (of which Peer Reviewed: 1 results,  Open Access: 1 results) Presentation (2 results) (of which Int'l Joint Research: 1 results) Book (1 results)

  • [Journal Article] Respiratory complications of Stevens-Johnson syndrome (SJS): 3 cases of SJS-induced obstructive bronchiolitis2020

    • Author(s)
      Kaneko Yoshiko、Seko Yurie、Sotozono Chie、Ueta Mayumi、Sato Satoshi、Shimamoto Takayuki、Iwasaku Masahiro、Yamada Tadaaki、Uchino Junji、Hizawa Nobuyuki、Takayama Koichi
    • Journal Title

      Allergology International

      Volume: 69 Issue: 3 Pages: 465-467

    • DOI

      10.1016/j.alit.2020.01.003

    • NAID

      130007877681

    • ISSN
      1323-8930, 1440-1592
    • Related Report
      2020 Research-status Report
    • Peer Reviewed / Open Access
  • [Presentation] Stevens-Johnson症候群(SJS)呼吸器合併症3症例の知られざる実態2020

    • Author(s)
      金子美子 瀬戸友利恵 外園千恵 杉本匠 菅佳史 齋ノ内玲 片山勇輝 田中里美 森本健司 嶋本貴之 岩破將博 山田忠明 内野順治 髙山浩一
    • Organizer
      第60回日本呼吸器学会総会 ( online開催)
    • Related Report
      2020 Research-status Report
  • [Presentation] Respiratory complications of Stevens-Johnson syndrome(SJS) 3 cases of SJS-induced obstructive bronchiolitis2020

    • Author(s)
      Yurie Seto, Yoshiko Kaneko, Chie Sotozono, Mayumi Ueta, Satoshi Sato, Hiroshi Date, Akihiko Yoshizawa, Takayuki Shimamoto, Masahiro Iwasaku, Tadaaki Yamada, Junji Uchino, Nobuyuki Hizawa, Koichi Takayama
    • Organizer
      JSA/WAO Joint Congress 2020 (online開催)
    • Related Report
      2020 Research-status Report
    • Int'l Joint Research
  • [Book] Derma. 皮膚アレルギー診療のすべて「重症薬疹の肺障害」2021

    • Author(s)
      森田栄伸
    • Total Pages
      242
    • Publisher
      全日本病院出版会
    • Related Report
      2021 Research-status Report

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Published: 2020-04-28   Modified: 2024-01-30  

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