Clinical and pathophysiological significance of novel identified anti-IFIH1/MDA5 autoantibody in amyopathic dermatomyositis

新型抗 IFIH1/MDA5 自身抗体在无肌病性皮肌炎中的临床和病理生理学意义

• 批准号: 22659185
• 负责人: MIMORI Tsuneyo
• 金额: $ 2.07万
• 依托单位: Kyoto University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Challenging Exploratory Research
• 财政年份: 2010
• 资助国家: 日本
• 起止时间: 2010 至 2011
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-22659185/
• 关键词: IFIH1; MDA5; CADM-140; 自己抗体; 皮膚筋炎; 間質性肺炎; サイトカイン; HLA; ADM

A disease-specific autoantibody, termed anti-CADM-140 antibody, is found in patients with amyopathic dermatomyositis accompanied with lethal acute interstitial pneumonia. We identified that the target autoantigen recognized by anti-CADM-140 antibody was IFIH1/MDA5, a viral RNA receptor in cytoplasm. The patients with anti-CADM-140 antibody revealed higher level of serum ferritin as well as IL-6, IL-10, IL-18 and M-CSF than antibody-negative dermatomyositis patients, suggesting the macrophage activating state. The intensive immunosuppressive therapy(high-dose glucocorticoids, cyclosporine and intravenous cyclophosphamide pulse) appeared to improve a life prognosis of antibody-positive patients when compared with the conventional step-up therapy.

一种疾病特异性自身抗体，称为抗CADM-140抗体，见于无肌病性皮肌炎伴致命性急性间质性肺炎患者。我们鉴定了抗CADM-140抗体识别的靶自身抗原是IFIH 1/MDA 5，一种细胞质中的病毒RNA受体。抗CADM-140抗体阳性皮肌炎患者血清铁蛋白、IL-6、IL-10、IL-18和M-CSF水平高于抗体阴性皮肌炎患者，提示巨噬细胞活化状态。强化免疫抑制治疗（高剂量糖皮质激素，环孢素和静脉环磷酰胺脉冲）出现改善抗体阳性患者的生活预后时，与传统的阶梯式治疗。

项目成果

自己抗体抗アミノアシルtRNA合成酵素抗体を検出するための新規ELISAの開発

开发新的 ELISA 检测自身抗体抗氨酰基-tRNA 合成酶抗体

• 发表时间: 2011
• 作者: 中嶋蘭;井村嘉孝;瀬戸美苗;村上昭弘;小林志緒;細野祐司;湯川尚一郎;吉藤元;川端大介;大村浩一郎;臼井崇;藤井隆夫;三森経世
• 通讯作者: 三森経世

Detection of antisynthetase syndrome in patients with idiopathic interstitial pneumonias

• DOI: 10.1016/j.rmed.2011.03.022
• 发表时间: 2011-08-01
• 期刊: RESPIRATORY MEDICINE
• 影响因子: 4.3
• 作者: Watanabe, Kizuku;Handa, Tomohiro;Mishima, Michiaki
• 通讯作者: Mishima, Michiaki

A New ELISA System for Detecting Autoantibodies to aminoacyl-tRNA Synthetases : Usefulness in Myositis and Interstitial Pneumonia.

用于检测氨酰-tRNA 合成酶自身抗体的新型 ELISA 系统：在肌炎和间质性肺炎中的有用性。

• 发表时间: 2010
• 作者: Ran Nakashima;Yoshitaka Imura;Shio Kobayashi;Yuji Hosono;Naoichiro Yukawa;Daisuke Kawabata;Takaki Nojima;Koichiro Ohmura;Takashi Usui;Takao Fujii;Minae Seto;Akihiro Murakami;Tsuneyo Mimori
• 通讯作者: Tsuneyo Mimori

Clinical and Pathogenic Significance of Autoantibodies in Autoimmune Myositis and Interstitial Lung Disease

自身抗体在自身免疫性肌炎和间质性肺疾病中的临床和致病意义

• 发表时间: 2011
• 作者: Takahashi K;Oka A;Mizuguchi M;Saitoh M;Takita J;Sato A;Mimaki M;Kato M;Ogawa S;Igarashi T;松井啓隆;Mimori T
• 通讯作者: Mimori T

HRCT features of interstitial lung disease in dermatomyositis with anti-CADM-140 antibody

• DOI: 10.1016/j.rmed.2011.05.006
• 发表时间: 2011-09-01
• 期刊: RESPIRATORY MEDICINE
• 影响因子: 4.3
• 作者: Tanizawa, Kiminobu;Handa, Tomohiro;Mishima, Michiaki
• 通讯作者: Mishima, Michiaki