Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
Pediatric Cardiology and Cardiac Surgery 38(3): 172-177 (2022)
doi:10.9794/jspccs.38.172

症例報告Case Report

重症筋無力症を合併した好酸球性心筋炎の幼児の一例An Infant Case of Eosinophilic Myocarditis with Myasthenia Gravis

榊原記念病院小児循環器科Department of Pediatric Cardiology, Sakakibara Heart Institute ◇ Tokyo, Japan

受付日:2021年11月15日Received: November 15, 2021
受理日:2022年5月19日Accepted: May 19, 2022
発行日:2022年8月1日Published: August 1, 2022
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好酸球性心筋炎(以下EMC)は,ステロイド治療が著効しより早い段階から導入されることで回復が見込める比較的予後良好とされる心筋疾患である.ステロイド漸減中に重症筋無力症(以下MG)が発症した症例報告はない.症例は3歳女児,先行感染がない急な顔色不良と呼吸苦症状で入院し,心臓超音波で壁運動低下を認めた.初期治療のステロイド終了後,第9病日より好酸球増多と心筋逸脱酵素の再上昇を認めたためEMCを疑い,ステロイド投与を再開した.好酸球数低下に伴い壁運動改善を認め,第81病日に退院した.その後筋力低下による歩行困難,右眼瞼下垂より精査を進めMGと診断され,ステロイド投与に加え免疫抑制剤を内服し軽快した.EMCを疑った場合には早期の段階よりPSLを導入することが重要である.またEMCとMGは共に免疫の賦活化が発症機序であり,両疾患の関連が推察された.このためステロイド漸減中の神経症状には注意が必要と考える.

Eosinophilic myocarditis (EMC) is a myocardial disease with a benign prognosis, for which patients are successfully treated by the early administration of steroids. To date, no studies have reported the occurrence of myasthenia gravis (MG) during tapering of the steroid dose. A 3-year-old girl was admitted to our hospital with pallor and in respiratory distress, which were not associated with an antecedent infection. Echocardiography displayed depression of the wall motion. We suspected EMC because of eosinophilia and increased cardiac enzymes after the initial steroid therapy, so steroid treatment was restarted. The cardiac wall motion improved thereafter, with a decrease in peripheral blood eosinophilic count. After discharge from our hospital, she developed gait difficulty with muscle weakness and right blepharoptosis, and after detailed examination, she was diagnosed with MG. She was treated with immunosuppressants in addition to steroids, and her MG symptoms disappeared. Thus, it is important to administer steroids at the early phase when EMC was suspected. MG related to an immunological mechanism may occur because immunity is involved in the onset of EMC. This case suggests attending to neurological symptoms during tapering of the steroid dose.

Key words: eosinophilic myocarditis; hypereosinophilic syndrome; myasthenia gravis; predonisolone; giant thrombus

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This page was last modified on 2022-11-11T09:20:17.000+09:00


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