IgG4関連動脈周囲炎/後腹膜線維症の臨床像の解析と本疾患に対する特異的診断基準  [in Japanese] Clinical and Pathological Characteristics of IgG4-related Periaortitis/Periarteritis and Retroperitoneal Fibrosis Diagnosed Based on Experts' Diagnosis  [in Japanese]

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Author(s)

    • 水島 伊知郎 Mizushima Ichiro
    • 金沢大学附属病院リウマチ・膠原病内科 Division of Rheumatology, Department of Cardiovascular and Internal Medicine, Kanazawa University Hospital
    • 松本 康 Matsumoto Yasushi
    • 金沢医療センター心臓血管外科 Department of Cardiovascular Surgery, National Hospital Organization, Kanazawa Medical Center
    • 網谷 英介 Amiya Eisuke
    • 東京大学医学部循環器内科 Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo
    • 山田 和徳 Yamada Kazunori
    • 金沢大学附属病院リウマチ・膠原病内科 Division of Rheumatology, Department of Cardiovascular and Internal Medicine, Kanazawa University Hospital
    • 堂本 裕加子 Domoto Yukako
    • 東京大学医学部人体病理学・病理診断学 Department of Pathology, Graduate School of Medicine, The University of Tokyo
    • 川野 充弘 Kawano Mitsuhiro
    • 金沢大学附属病院リウマチ・膠原病内科 Division of Rheumatology, Department of Cardiovascular and Internal Medicine, Kanazawa University Hospital
    • 全 陽 Zen Yoh
    • 神戸大学医学部病理部 Diagnostic Pathology, Kobe University Graduate School of Medicine
    • 井上 大 Inoue Dai
    • 金沢大学医学部放射線科 Department of Radiology, Kanazawa University Graduate School of Medicine
    • 山本 元久 Yamamoto Motohisa
    • 札幌医科大学医学部免疫・リウマチ内科学 Department of Rheumatology and Clinical Immunology, Sapporo Medical University
    • 笠島 史成 Kasashima Fuminari
    • 金沢医療センター心臓血管外科 Department of Cardiovascular Surgery, National Hospital Organization, Kanazawa Medical Center

Abstract

<p>エキスパートによりIgG4関連の大動脈周囲炎/動脈周囲炎および後腹膜線維症と診断された99例のデータを解析し,臨床像や診断に至った経緯を検討した。33例は動脈周囲/後腹膜の病理組織学的所見から,50例は他臓器のIgG4関連疾患の確診から,残りの16例は臨床経過などから総合的に診断されていた。IgG4関連疾患の厚生労働省研究班により策定された新しい臓器特異的診断基準を用いた検討では,73例(73.7%)が確診,6例(6.1%)が準確診,17例(17.2%)が疑診に分類された。</p>

<p>IgG4-related disease is a systemic disease, characterized by elevation of serum IgG4 and, histopathologically, massive infiltration of IgG4+ lymphocyte and plasma cell infiltration, storiform fibrosis, causing enlargement, nodules or thickening. It may affect various organs simultaneously or metachronously. Here we analyzed the clinical and pathological characteristics of 99 patients diagnosed with IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis. Of 99 patients (women/men, 15/84; mean age 67.3±9.5 years), 33 were diagnosed based on the histopathological findings of perivascular/retroperitoneal lesions, 50 were diagnosed based on the characteristic imaging findings of perivascular/retroperitoneal lesions and the presence of definitive IgG4-related disease in other organ(s), and the remaining 16 patients were diagnosed by experts based on the characteristic imaging findings of perivascular/retroperitoneal legions, serological findings, response to glucocorticoid treatment, and/or the presence of suspected IgG4-related disease in other organ(s). According to the new organ-specific criteria proposed by experts, 73 (73.7%) diagnoses were categorized to be definitive, and 6 (6.1%) and 17 (17.2%) diagnoses were categorized to be probable and possible, respectively. Further analyses are needed to clarify the optimal diagnostic and therapeutic strategy of IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis.</p>

Journal

  • The Journal of Japanese College of Angiology

    The Journal of Japanese College of Angiology 58(8), 117-129, 2018

    Japanese College of Angiology

Codes

  • NII Article ID (NAID)
    130007432232
  • Text Lang
    JPN
  • ISSN
    0387-1126
  • Data Source
    J-STAGE 
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