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心肌炎的谱:从病理学到临床。

The spectrum of myocarditis: from pathology to the clinics.

机构信息

Cardiovascular Pathology Unit, Department of Pathology, Sant'Orsola-Malpighi University Hospital, Via G. Massarenti 9, 40138, Bologna, Italy.

Cardiovascular Department, San Donato Hospital, Arezzo, Italy.

出版信息

Virchows Arch. 2019 Sep;475(3):279-301. doi: 10.1007/s00428-019-02615-8. Epub 2019 Jul 11.

Abstract

Myocarditis is an inflammatory disease of the myocardium, which may occur in isolation or as part of systemic infectious/immune/autoimmune conditions, characterized by vast aetiologic, clinical and histopathologic heterogeneity. The broad spectrum of myocarditis can be categorized according to the prevalent histopathologic pattern including lymphocytic, lympho-histiocytic, eosinophilic and neutrophilic forms, giant cell myocarditis and myocarditis with granulomata. Diverse histopathologic substrates generally reflect different aetiologies and pathogenetic mechanisms and may be critical to clinical decision-making. Active vasculitis, when present, completes the inflammatory spectrum. Unfortunately, the correlation of histopathologic patterns, clinical presentation and disease course in myocarditis is still largely unresolved, due to limited availability of bioptic samples at specific stages of disease and impracticality of serial sampling. We here review the elements supporting an aetiology-driven diagnostic work-up in myocarditis, emphasizing the importance of integrating pathologic studies with clinical features and information derived from multimodality imaging. Furthermore, we explore myocardial inflammation in genetic cardiomyopathies, its role in driving clinical variability and the potential of transcriptomic and proteomic analysis in our understanding of these complex interrelations.

摘要

心肌炎是心肌的炎症性疾病,可单独发生或作为全身感染/免疫/自身免疫性疾病的一部分,其特征为广泛的病因、临床和组织病理学异质性。心肌炎的广泛谱可根据流行的组织病理学模式进行分类,包括淋巴细胞性、淋巴组织细胞性、嗜酸性粒细胞性和中性粒细胞性、巨细胞性心肌炎和伴有肉芽肿的心肌炎。不同的组织病理学基质通常反映不同的病因和发病机制,可能对临床决策至关重要。如果存在活动性脉管炎,则完成炎症谱。不幸的是,由于在疾病的特定阶段获取活检样本的可用性有限,以及连续取样不切实际,心肌炎中组织病理学模式、临床表现和疾病过程之间的相关性在很大程度上仍未得到解决。我们在此回顾了支持心肌炎病因驱动诊断评估的要素,强调了将病理学研究与临床特征和来自多模态成像的信息相结合的重要性。此外,我们探讨了遗传性心肌病中的心肌炎症,其在驱动临床变异性中的作用,以及转录组学和蛋白质组学分析在理解这些复杂相互关系中的潜力。

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