{"@context":{"@vocab":"https://cir.nii.ac.jp/schema/1.0/","rdfs":"http://www.w3.org/2000/01/rdf-schema#","dc":"http://purl.org/dc/elements/1.1/","dcterms":"http://purl.org/dc/terms/","foaf":"http://xmlns.com/foaf/0.1/","prism":"http://prismstandard.org/namespaces/basic/2.0/","cinii":"http://ci.nii.ac.jp/ns/1.0/","datacite":"https://schema.datacite.org/meta/kernel-4/","ndl":"http://ndl.go.jp/dcndl/terms/","jpcoar":"https://github.com/JPCOAR/schema/blob/master/2.0/"},"@id":"https://cir.nii.ac.jp/crid/1390282679989648640.json","@type":"Article","productIdentifier":[{"identifier":{"@type":"DOI","@value":"10.3950/jibiinkoka.119.1475"}},{"identifier":{"@type":"NDL_BIB_ID","@value":"027831456"}},{"identifier":{"@type":"URI","@value":"http://id.ndl.go.jp/bib/027831456"}},{"identifier":{"@type":"URI","@value":"https://ndlsearch.ndl.go.jp/books/R000000004-I027831456"}},{"identifier":{"@type":"URI","@value":"https://www.jstage.jst.go.jp/article/jibiinkoka/119/12/119_1475/_pdf"}},{"identifier":{"@type":"NAID","@value":"130005290546"}}],"dc:title":[{"@language":"ja","@value":"他科に学ぶ自己免疫疾患"},{"@value":"他領域からのトピックス 他科に学ぶ自己免疫疾患 : IgG4関連疾患"},{"@language":"ja-Kana","@value":"タ リョウイキ カラ ノ トピックス タ カ ニ マナブ ジコ メンエキ シッカン : IgG4 カンレン シッカン"}],"dcterms:alternative":[{"@language":"ja","@value":"IgG4 関連疾患"}],"dc:language":"ja","description":[{"type":"abstract","notation":[{"@language":"ja","@value":"<p>　IgG4 関連疾患とは IgG4 が関連する全身性疾患であり, 最近確立された疾患概念である. 臨床的特徴は, ① 病変が全身に分布し, これらの多くは従来その臓器独自の病名で診断治療されてきた, ② 画像所見として腫大, 結節, 壁肥厚を呈する, ③ 血中 IgG4 値が通常 135mg/dl 以上である, ④ 病変局所にリンパ球形質細胞浸潤, IgG4 陽性形質細胞浸潤を認める, ⑤ ステロイド治療に良好に反応する, ⑥ 他の IgG4 関連疾患を同時性, 異時性に合併することが多い, に集約される. 本疾患概念成立には, 自己免疫性膵炎で血中 IgG4 値が高率, 特異的に上昇し, 病変組織に IgG4 陽性形質細胞が特異的に浸潤すること, が明らかになったことが大きく貢献している. IgG4 関連疾患はほぼ全身諸臓器に分布しているが, 代表的構成疾患は IgG4 関連涙腺・唾液腺炎 (ミクリッツ病), IgG4 関連呼吸器病変, 自己免疫性膵炎, IgG4 関連硬化性胆管炎, IgG4 関連後腹膜線維症, IgG4 関連腎病変などがある. 耳鼻咽喉科領域の IgG4 関連疾患として IgG4 関連ミクリッツ病と IgG4 関連キュットナー腫瘍が知られているが, 新しい疾患概念の可能性として IgG4 関連鼻副鼻腔炎が提唱されている. IgG4 関連疾患の診断は「IgG4 関連疾患包括診断基準2011」によるが, 悪性疾患との鑑別が肝要である. IgG4 関連疾患は長期経過で, 機能障害を呈する慢性期の病態への移行, 悪性腫瘍の合併などが想定され, 今後の検討課題である.</p>"}],"abstractLicenseFlag":"disallow"}],"creator":[{"@id":"https://cir.nii.ac.jp/crid/1420845751158065280","@type":"Researcher","personIdentifier":[{"@type":"KAKEN_RESEARCHERS","@value":"10177628"},{"@type":"NRID","@value":"1000010177628"},{"@type":"NDL_AUTHOR_ID","@value":"01191403"},{"@type":"NRID","@value":"9000412638922"},{"@type":"NRID","@value":"9000412637614"},{"@type":"NRID","@value":"9000021202835"},{"@type":"NRID","@value":"9000392640909"},{"@type":"NRID","@value":"9000258013382"},{"@type":"NRID","@value":"9000415232085"},{"@type":"NRID","@value":"9000412582403"},{"@type":"NRID","@value":"9000001337607"},{"@type":"NRID","@value":"9000107385446"},{"@type":"NRID","@value":"9000412638320"},{"@type":"NRID","@value":"9000406403299"},{"@type":"NRID","@value":"9000001022208"},{"@type":"NRID","@value":"9000002019910"},{"@type":"NRID","@value":"9000402293216"},{"@type":"NRID","@value":"9000412638635"},{"@type":"NRID","@value":"9000021321514"},{"@type":"NRID","@value":"9000403303013"},{"@type":"NRID","@value":"9000415172366"},{"@type":"NRID","@value":"9000412605683"},{"@type":"NRID","@value":"9000412605649"},{"@type":"NRID","@value":"9000001576984"},{"@type":"NRID","@value":"9000412638952"},{"@type":"NRID","@value":"9000414837636"},{"@type":"NRID","@value":"9000415202176"},{"@type":"NRID","@value":"9000412605686"},{"@type":"NRID","@value":"9000389551750"},{"@type":"NRID","@value":"9000283271775"},{"@type":"NRID","@value":"9000412626810"},{"@type":"NRID","@value":"9000018484466"},{"@type":"NRID","@value":"9000022057961"},{"@type":"NRID","@value":"9000240058601"},{"@type":"NRID","@value":"9000263064228"},{"@type":"NRID","@value":"9000412637742"},{"@type":"NRID","@value":"9000412637700"},{"@type":"NRID","@value":"9000412626414"},{"@type":"NRID","@value":"9000021433684"},{"@type":"NRID","@value":"9000022069632"},{"@type":"NRID","@value":"9000243885348"},{"@type":"NRID","@value":"9000265249499"},{"@type":"NRID","@value":"9000412638894"},{"@type":"NRID","@value":"9000412605660"},{"@type":"NRID","@value":"9000021942552"},{"@type":"NRID","@value":"9000351065773"},{"@type":"NRID","@value":"9000405873480"},{"@type":"NRID","@value":"9000020865511"},{"@type":"NRID","@value":"9000363332847"},{"@type":"NRID","@value":"9000018556703"},{"@type":"NRID","@value":"9000412638181"},{"@type":"NRID","@value":"9000412104013"},{"@type":"NRID","@value":"9000412605672"},{"@type":"NRID","@value":"9000412607030"},{"@type":"NRID","@value":"9000415162689"},{"@type":"NRID","@value":"9000412637606"},{"@type":"NRID","@value":"9000403402392"},{"@type":"NRID","@value":"9000021930946"},{"@type":"NRID","@value":"9000021264178"},{"@type":"NRID","@value":"9000021162656"},{"@type":"NRID","@value":"9000022129003"},{"@type":"RESEARCHMAP","@value":"https://researchmap.jp/read0010391"}],"foaf:name":[{"@language":"ja","@value":"川 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