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心肌结节病:诊断与管理。

Cardiac sarcoidosis: diagnosis and management.

机构信息

Pulmonary Department, Corfu General Hospital, Corfu 49100, Greece.

出版信息

Rev Cardiovasc Med. 2020 Sep 30;21(3):321-338. doi: 10.31083/j.rcm.2020.03.102.

Abstract

Sarcoidosis is a chronic inflammatory disease of unknown etiology characterized by multi-organ involvement. End-organ disease consists of granulomatous inflammation, which if left untreated or not resolved spontaneously, leads to permanent fibrosis and end-organ dysfunction. Cardiac involvement and fibrosis in sarcoidosis occur in 5-10% of cases and is becoming increasingly diagnosed. This is due to increased clinical awareness among clinicians and new diagnostic modalities, since magnetic resonance imaging and positron-emission tomography are emerging as "gold standard" tools replacing endomyocardial biopsy. Despite this progress, isolated cardiac sarcoidosis is difficult to differentiate from other causes of arrhythmogenic cardiomyopathy. Cardiac fibrosis leads to congestive heart failure, arrhythmias and sudden cardiac death. Immunosuppressives (mostly corticosteroids) are used for the treatment of cardiac sarcoidosis. Implantable devices like a cardioverter-defibrillator may be warranted in order to prevent sudden cardiac death. In this article current trends in the pathophysiology, diagnosis and management of cardiac sarcoidosis will be reviewed focusing on published research and latest guidelines. Lastly, a management algorithm is proposed.

摘要

结节病是一种病因不明的慢性炎症性疾病,其特征为多器官受累。终末器官疾病由肉芽肿性炎症组成,如果未经治疗或未自发消退,会导致永久性纤维化和终末器官功能障碍。结节病的心脏受累和纤维化在 5-10%的病例中发生,且发病率越来越高。这是由于临床医生的临床意识提高和新的诊断方式,因为磁共振成像和正电子发射断层扫描作为替代心内膜心肌活检的“金标准”工具而出现。尽管取得了这些进展,但孤立性心脏结节病仍难以与其他致心律失常性心肌病的病因区分。心脏纤维化可导致充血性心力衰竭、心律失常和心源性猝死。免疫抑制剂(主要是皮质类固醇)用于治疗心脏结节病。为了预防心源性猝死,可能需要植入心脏转复除颤器等植入式设备。本文将重点讨论已发表的研究和最新指南,综述心脏结节病的病理生理学、诊断和治疗的最新进展。最后提出了一个管理算法。

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