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Japanese

Title 小児リウマチ性疾患
Subtitle 特集 小児重症病態治療におけるアフェレシス治療の位置づけ 総説
Authors 清水正樹
Authors (kana)
Organization 東京医科歯科大学大学院医歯学総合研究科 小児地域成育医療学講座
Journal 日本アフェレシス学会雑誌
Volume 39
Number 3
Page 160-168
Year/Month 2020 /
Article 報告
Publisher 日本アフェレシス学会
Abstract 「1. はじめに」近年リウマチ性疾患に対する治療は, 生物学的製剤による分子標的治療の発展により劇的ともいえる飛躍的な進歩を遂げ, その予後も著明に改善してきている. しかしながらその一方で, リウマチ性疾患に続発する二次性の血球貪食性リンパ組織球症(hemophagocytic lymphohistiocytosis:HLH)であるマクロファージ活性化症候群(macrophage activation syndrorne:MAS)や若年性皮膚筋炎(juvenile dermatomyositis)に合併する急速進行性間質性肺障害(rapidly progressive interstitial lung disease:RP-ILD)のような重症の難治性症例も依然存在する. このような症例において, 病態に深く関与している炎症性サイトカイン, 病原性自己抗体, 免疫複合体などの病因物質, あるいは炎症細胞を直接除去して病態を改善させるアフェレシス治療の有効性を示唆する報告は多く, その存在意義は依然大きい.
Practice 医学一般
Keywords apheresis, juvenile idiopathic arthritis, systemic lupus erythematosus, juvenile dermatomyositis

English

Title Therapeutic Apheresis for Pediatric Rheumatic Diseases
Subtitle
Authors Masaki Shimizu
Authors (kana)
Organization Department of Child Health and Development, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
Journal Japanese Journal of Apheresis
Volume 39
Number 3
Page 160-168
Year/Month 2020 /
Article Report
Publisher Japanese Society for Apheresis
Abstract [Summary] Recently, various biologics have been developed. These drugs show a dramatic therapeutic effect for pediatric rheumatic diseases. However, it is still challenging to treat refractory diseases such as macrophage activation syndrome associated with systemic juvenile idiopathic arthritis (JIA), thrombotic microangiopathy in systemic lupus erythematosus (SLE), catastrophic antiphospholipid syndrome, and rapidly progressive interstitial lung diseases associated with anti-melanoma differentiation-associated gene positive juvenile dermatomyositis (JDM). Apheresis which can directly remove pathogenic agents including proinflammatory cytokines, autoantibodies, immune complexes and inflammatory cells has a significant therapeutic role in combination with immunosuppressive treatment. Here, I review the treatment strategies and the role of apheresis for JIA, SLE and JDM.
Practice General medicine
Keywords apheresis, juvenile idiopathic arthritis, systemic lupus erythematosus, juvenile dermatomyositis
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