Idiopathic giant cell myocarditis or cardiac sarcoidosis? A retrospective audit of a nationwide case series
Idiopathic giant cell myocarditis or cardiac sarcoidosis? A retrospective audit of a nationwide case series
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DOI：
10.1002/ehf2.12725
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发表时间：
2020-04-28
影响因子：
3.8
通讯作者：
Kupari, Markku
中科院分区：
文献类型：
--
作者：
Ekstrom, Kaj;Raisanen-Sokolowski, Anne;Kupari, Markku
心脏结节病（CS）和巨细胞心肌炎（GCM）是两种具有共同病理和临床特征的炎症性心肌病。它们的区别是困难的，容易混淆和明显的错误。他们代表不同的表型的一个单一的diseases.Methods和结果的可能性，我们做了一个回顾性的审计，自20世纪80年代末在芬兰诊断的73例GCM。重新分析所有可用的组织学材料，以及与GCM和CS之间的区别相关的其他检查。在心脏内或心脏外发现肉芽肿被认为是CS的诊断，不包括GCM。73例GCM中共有45例（62%）被重新分类为CS。除1例病例外，所有病例均基于发现结节样肉芽肿，这些结节样肉芽肿要么最初被遗漏（n = 29），要么被误解（n = 11），要么在额外的移植后心肌标本（n = 3）或病程中累积的心外组织样本（n = 1）中发现。支持重新分类，患者搬迁到CS组有较少的心力衰竭在介绍（患病率20%与46%，P = 0.017）和更好的1年无移植生存率（82%与45%，P = 0.011）比患者被认为是代表真正的GCM.Conclusions识别肉芽肿的心脏内外仍然是一个挑战的病理学家。考虑到CS和GCM被认为是GCM之外的不同疾病和肉芽肿，许多GCM病例，如果彻底检查，可能需要重新分类为CS。然而，CS和GCM是否是真正不同的实体或一个疾病连续体的一部分尚未得到最终解决。
Aims Cardiac sarcoidosis (CS) and giant cell myocarditis (GCM) are inflammatory cardiomyopathies sharing histopathological and clinical features. Their differentiation is difficult and susceptible of confusion and apparent mistakes. The possibility that they represent different phenotypes of a single disease has been debated.Methods and results We made a retrospective audit of 73 cases of GCM diagnosed in Finland since the late 1980s. All available histological material was reanalyzed as were other examinations pertinent to the distinction between GCM and CS. Finding granulomas in or outside the heart was considered diagnostic of CS and exclusive of GCM. Altogether 45 of the 73 cases of GCM (62%) were reclassified as CS. In all except one case, this was based on finding sarcoid granulomas that either had been originally missed (n = 29) or misinterpreted (n = 11) or were found in additional posttransplant myocardial specimens (n = 3) or samples of extracardiac tissue (n = 1) accrued over the disease course. Supporting the reclassification, patients relocated to the CS group had less heart failure at presentation (prevalence 20% vs. 46%, P = 0.017) and better 1 year transplant-free survival (82% vs. 45%, P = 0.011) than patients considered to represent true GCM.Conclusions Recognizing granulomas in or outside the heart remains a challenge for the pathologist. Given that CS and GCM are considered distinct diseases and granulomas exclusive of GCM, many cases of GCM, if thoroughly scrutinized, may need reclassification as CS. However, whether CS and GCM are truly different entities or parts of a one-disease continuum has not yet been conclusively settled.