Cardiac complication of myositis associated with anti-mitochondrial antibodies

• Project/Area Number: 25860731
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Neurology
• Research Institution: Okinaka Memorial Institute for Medical Research
• Principal Investigator: MAEDA MEIKO 公益財団法人冲中記念成人病研究所, その他部局等, 研究員 (20646799)
• Research Collaborator: Shimizu Jun 東京大学, 附属病院神経内科, 准教授 ; Uesaka Yoshikazu 国家公務員共済組合連合会, 虎の門病院神経内科, 部長
• Project Period (FY): 2013-04-01 – 2017-03-31
• Project Status: Completed (Fiscal Year 2016)
• Budget Amount: ¥3,510,000 (Direct Cost: ¥2,700,000、Indirect Cost: ¥810,000); Fiscal Year 2016: ¥780,000 (Direct Cost: ¥600,000、Indirect Cost: ¥180,000); Fiscal Year 2015: ¥780,000 (Direct Cost: ¥600,000、Indirect Cost: ¥180,000); Fiscal Year 2014: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000); Fiscal Year 2013: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
• Keywords: 筋炎 / 自己抗体 / 心臓合併症 / 呼吸筋合併症 / 抗ミトコンドリア抗体 / 不整脈 / 原発性胆汁性肝硬変

Outline of Final Research Achievements

Eighteen AMA-positive myositis patients with cardiac complications (11 females and 7 males) were identified. The ages at onset of symptom ranged from 36 to 70 years (mean, 50.1 ± 9.0 years). Duration between the onsets of symptom to end of follow-up (disease courses) ranged 1.5 to 18.0 years (mean, 9.0 ± 5.2 years). All patients were associated with arrhythmia and had decreased EF ranging 9 to 71 % (mean, 40.2 ± 17.2 %). Ten patients required intervention therapy (catheter ablation, n=7; defibrillator, n=3; pacemaker, n=2; mitral valve surgery, n=1). Four patients died in the follow-up period including two patients who died suddenly. Histopathological analysis revealed that the biopsied specimen of all the patients fulfilled the criteria of non-specific myositis.

Research Products

• [Journal Article] Sporadic late-onset nemaline myopathy as a rare cause of slowly progressive muscle weakness with young adult onset (2015)
  • Author(s): Maeda MH, Ohta H, Izutsu K, Shimizu J, Uesaka Y
  • Journal Title: Muscle Nerve Volume: 00 Issue: 5 Pages: 00-00
  • DOI: 10.1002/mus.24509
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] Anti-mitochondrial antibody: Potential marker of myositis with chronic clinical course, muscle atrophy, cardiac involvement and granulomatous inflammation in muscle biopsy (2013)
  • Author(s): Meiko Hashimoto Maeda and Jun Shimizu
  • Journal Title: Clinical and Experimental Neuroimmunology Volume: 4 Issue: 1 Pages: 18-18
  • DOI: 10.1111/cen3.12026
  • Peer Reviewed
• [Presentation] 免疫介在性と考えられた血液内科疾患移植後末梢神経障害の生検例に関する検討 (2015)
  • Author(s): 前田 明子、神崎 真実、上坂 義和、谷口 修一、清水 潤
  • Organizer: 第27回神経免疫学会学術集会
  • Place of Presentation: 岐阜
  • Year and Date: 2015-09-15
• [Presentation] Serum cytokine analysis in idiopathic inflammatory myopathies diagnosed on the basis of pathological findings (2014)
  • Author(s): Meiko Hashimoto Maeda Yoshikazu Uesaka, Shoji Tsuji, Jun Shimizu
  • Organizer: American Academy of Neurology annual meeting
  • Place of Presentation: Philadelphia
  • Year and Date: 2014-04-26 – 2014-05-03
• [Presentation] Serum cytokine analysis in idiopathic inflammatory myopathies diagnosed on the basis of pathological findings (2014)
  • Author(s): Meiko Hashimoto Maeda, Yoshikazu Uesaka, Shoji Tsuji, Jun Shimizu
  • Organizer: 66th American Academy of Neurology annual meeting
  • Place of Presentation: Philadelphia
• [Presentation] 病理所見で分類した筋炎グループにおける血清液性因子に関する検討 (2014)
  • Author(s): 前田明子, 肥田あゆみ, 清水 潤
  • Organizer: 平成25年度 免疫神経疾患に関する調査研究班 班会議
  • Place of Presentation: 東京
• [Presentation] 抗ミトコンドリア抗体陽性/原発性胆汁性肝硬変合併筋炎での心臓合併症に関する検討 (2013)
  • Author(s): 前田明子, 肥田あゆみ, 上坂義和, 清水 潤
  • Organizer: 第54回日本神経学会総会
  • Place of Presentation: 東京
• [Presentation] 多発筋炎の診断基準、希少筋炎の病理に関して (2013)
  • Author(s): 前田 明子
  • Organizer: 第一回姫路免疫性神経疾患研究会
  • Place of Presentation: 姫路
  • Invited
• [Presentation] 抗ミトコンドリア抗体陽性筋炎症例の心合併症の特徴に関する検討 (2013)
  • Author(s): 前田明子, 肥田あゆみ, 上坂義和, 清水 潤
  • Organizer: 第25回日本神経免疫学会学術集会
  • Place of Presentation: 下関
• [Book] 第4版 EBMに基づく脳神経疾患の基本治療指針 (2016)
  • Author(s): 前田 明子
  • Total Pages: 807
  • Publisher: メジカルビュー
• [Book] 脳卒中症候学 症例編 診療の深みを理解する (2016)
  • Author(s): 前田 明子、武田 克彦
  • Publisher: 西村書店
• [Book] 最新医学社別冊 新しい診断と治療のABC81 多発性筋炎・皮膚筋炎 (2014)
  • Author(s): 前田明子，清水潤
  • Total Pages: 15
  • Publisher: 最新医学社
• [Book] 神経内科 (2013)
  • Author(s): 前田明子，清水潤
  • Total Pages: 9
  • Publisher: 科学評論社
• [Remarks] 公益財団法人冲中記念成人病研究所 今月の研究者 前田明子
  • URL: http://okiken.tokyo/scientist/index.php?id=11