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积极免疫抑制治疗成功治愈暴发性心脏结节病

Fulminant Cardiac Sarcoidosis Successfully Treated With Aggressive Immunosuppressive Therapy.

作者信息

Yasumura Kaori, Sera Fusako, Akazawa Yasuhiro, Nakamoto Kei, Kawai Makiko, Kurashige Masako, Nakamura Daisuke, Oka Takafumi, Mizote Isamu, Morii Eiichi, Ohtani Tomohito, Sakata Yasushi

机构信息

Department of Cardiovascular Medicine, Osaka University Graduate School of Medicine, Suita, Japan.

Department of Pathology, Osaka University Graduate School of Medicine, Suita, Japan.

出版信息

Case Rep Cardiol. 2025 Mar 11;2025:1350557. doi: 10.1155/cric/1350557. eCollection 2025.

Abstract

The clinical course of cardiac sarcoidosis is typically subacute, and fulminant cases requiring mechanical circulatory support are rare. Here, we report the case of a patient with pathologically diagnosed cardiac sarcoidosis who presented with fulminant myocarditis and whose cardiac function was improved by aggressive immunosuppressive therapy based on the treatment of giant cell myocarditis. A 55-year-old woman presented with progressive dyspnoea and nausea that persisted for 1 month and was eventually diagnosed with acute heart failure. Echocardiography showed a reduced left ventricular ejection fraction with thinning of the basal septal wall. During hospitalisation, she experienced ventricular tachycardia and fibrillation attacks, and bradycardia due to a complete atrioventricular block and sinus dysfunction was observed after starting amiodarone. Subsequently, she underwent intra-aortic balloon pump insertion in addition to inotropic agent administration; however, venoarterial extracorporeal membrane oxygenation and Impella 5.0 were needed because biventricular dysfunction progressed. We diagnosed our patient with cardiac sarcoidosis based on the pathological findings revealing inflammatory cell infiltration, including giant cells with extensive fibrosis and granulomas. However, the possibility of giant cell myocarditis could not be ruled out because of the fulminant clinical course; therefore, aggressive immunosuppressive therapy with corticosteroids and cyclosporine was started. Her cardiac function improved, and all mechanical circulatory support and inotropic agents were discontinued. Cardiac sarcoidosis is difficult to differentiate from giant cell myocarditis because they have many similarities in terms of myocardial histopathology and clinical manifestations. While whether the two diagnoses are parts of a single-disease continuum remains debatable, aggressive combination immunosuppressive therapy may contribute to favourable outcomes.

摘要

心脏结节病的临床病程通常为亚急性,需要机械循环支持的暴发性病例很少见。在此,我们报告一例经病理诊断为心脏结节病的患者,该患者表现为暴发性心肌炎,基于巨细胞心肌炎的治疗方案,积极的免疫抑制治疗改善了其心功能。一名55岁女性出现进行性呼吸困难和恶心,持续1个月,最终被诊断为急性心力衰竭。超声心动图显示左心室射血分数降低,基底间隔壁变薄。住院期间,她经历了室性心动过速和颤动发作,开始使用胺碘酮后出现因完全性房室传导阻滞和窦性功能障碍导致的心动过缓。随后,除给予正性肌力药物外,她还接受了主动脉内球囊泵置入术;然而,由于双心室功能恶化,需要进行静脉-动脉体外膜肺氧合和Impella 5.0治疗。根据病理检查发现炎症细胞浸润,包括伴有广泛纤维化的巨细胞和肉芽肿,我们诊断该患者为心脏结节病。然而,由于临床病程暴发性,不能排除巨细胞心肌炎的可能性;因此,开始使用皮质类固醇和环孢素进行积极的免疫抑制治疗。她的心功能得到改善,所有机械循环支持和正性肌力药物均停用。心脏结节病很难与巨细胞心肌炎区分开来,因为它们在心肌组织病理学和临床表现方面有许多相似之处。虽然这两种诊断是否属于单一疾病的连续体仍有争议,但积极的联合免疫抑制治疗可能有助于取得良好的结果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0525/11986919/a095cad1d995/CRIC2025-1350557.001.jpg

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