Departmental Bulletin Paper IgG4 関連血管病変
IgG4-related vascular disease

笠島, 里美  ,  Kasashima, Satomi

41 ( 2 )  , pp.1 - 7 , 2018-01-31 , Wellness and Health Care Society , ウェルネス・ヘルスケア学会
IgG4 関連疾患(IgG4-relared disease; IgG4-RD)は,血清の IgG4 高値,組織での多数の IgG4陽性の形質細胞浸潤と線維増生を特徴とする疾患群である . 発見から 10 年以上経過し,全身ほぼ全ての臓器に発生しうる事や免疫異常が病因に関わる事等など,病態の理解も進んできた. 腹部大動脈瘤の一亜型に,動脈壁肥厚や炎症細胞浸潤が特徴的な炎症性腹部大動脈瘤 がある.IgG4-RD が多中心性の繊維増殖性疾患の視点から, 我々は炎症性腹部大動脈瘤の約半数が IgG4-RD である事を解明し,臨床病理像をまとめてきた . その後,心血管領域での IgG4-RD の探索を進め,大血管のみならず中型血管,冠動脈,大腿動脈等の末梢血管にまで発生することや,その多くが炎症性動脈瘤或いは動脈周囲炎を呈することも解明し,現在では1gG4 関連血管病変の概念が確立してきた . 本稿では,頻度が高く,prototype となる IgG4 関連炎症性腹部大動脈瘤から説明し, 胸部大動脈,中型動脈,心病変などについても,疾患概念,診断,臨床像,病理像,治療等の概略を示す.
IgG4-related disease (IgG4-RD) is a new disease entity characterized by serum IgG4 elevation, and pathological IgG4-positive plasma cell infiltration and fibrosis in the affected organs. It occurs in almost all organs of the whole body and its pathogenesis is associated with immune disorder.Inflammatory abdominal aortic aneurysm (IAAA) is a special subtype of abdominal aortic aneurysm characterized by arterial wall thickening and inflammatory cell infiltration. From the viewpoint of multicentric fibrous proliferative diseases, we focused on the histological similarity of idiopathic retroperitoneal fibrosis and IAAA, and about half of all cases of IAAA were clarified as IgG4-RD. We have estimated the clinicopathological characteristics of IgG4-RD occurring in the cardiovascular organs, not only in large vessels but also in peripheral blood vessels, such as medium-sized blood vessels, coronary artery, and femoral artery, etc. In addition, we showed that IgG4-RD occurred in the cardiovascular organs seen morphologically as inflammatory aneurysms or periarteritis, which were tumorous lesions surrounding the aorta or artery. This article first discusses IgG4-related IAAA, because of it was the just prototype of IgG4-RD in vascular organs, and then presents an outline of the disease concept, diagnosis, clinicopathological features, pathology, and treatment, in the thoracic aorta, middle-level arteries, cardiac lesions, etc.

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