Diagnosis, management, and outcome of cardiac sarcoidosis and giant cell myocarditis: a Swedish single center experience.
Diagnosis, management, and outcome of cardiac sarcoidosis and giant cell myocarditis: a Swedish single center experience.
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DOI：
10.1186/s12872-022-02639-0
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发表时间：
2022-04-26
影响因子：
2.1
通讯作者：
中科院分区：
文献类型：
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心脏结节病(CS)和巨细胞性心肌炎(GCM)是一种罕见的疾病，它们既有相似之处，又表现出不同的临床和组织病理学特征。我们的目的是比较被诊断为CS或GCM的患者的人口学特征、临床表现和结果。我们比较了1991年至2020年在我们中心诊断为CS(n = 71)或GCM(n = 21)的所有成人患者的临床数据和结果。CS和GCM患者的中位(四分位数范围)随访时间分别为33.5[6.5-60.9]个月和2.98[0.6-40.9]个月。在整个队列中，心力衰竭(HF)是最常见的表现(31%)，其次是室性心律失常(25%)。发病时，54%的CS患者的左心室射血分数为 <； 为50%，而86%的糖耐量低减患者为86%(P = 0.014)，而右室功能不全的相应比例分别为24%和52%(P = 0.026)。晚期心衰(NYHA ≥ IIIB)在CS(31%)中的发生率低于GCM(76%)。CS患者血中钠尿肽(P <； 0.001)和肌钙蛋白(P = 0.014)水平显著降低。在纳入生存分析的CS患者中，18%达到了死亡或心脏移植(HTX)的复合终点，相比之下，GCM患者的这一比例为68%(P <； 0.001)。GCM的临床病程比CS更剧烈，有严重的双室衰竭，循环生物标记物水平更高，对HTX的需求增加。即使在调整了心功能不全的标记物后，组织病理学诊断仍然是关键的决定因素。网上版载有补充材料，可在10.1186/s12872-022-02639-0查阅。
Cardiac sarcoidosis (CS) and giant cell myocarditis (GCM) are rare diseases that share some similarities, but also display different clinical and histopathological features. We aimed to compare the demographics, clinical presentation, and outcome of patients diagnosed with CS or GCM. We compared the clinical data and outcome of all adult patients with CS (n = 71) or GCM (n = 21) diagnosed at our center between 1991 and 2020. The median (interquartile range) follow-up time for patients with CS and GCM was 33.5 [6.5–60.9] and 2.98 [0.6–40.9] months, respectively. In the entire cohort, heart failure (HF) was the most common presenting manifestation (31%), followed by ventricular arrhythmias (25%). At presentation, a left ventricular ejection fraction of < 50% was found in 54% of the CS compared to 86% of the GCM patients (P = 0.014), while corresponding proportions for right ventricular dysfunction were 24% and 52% (P = 0.026), respectively. Advanced HF (NYHA ≥ IIIB) was less common in CS (31%) than in GCM (76%). CS patients displayed significantly lower circulating levels of natriuretic peptides (P < 0.001) and troponins (P = 0.014). Eighteen percent of patients with CS included in the survival analysis reached the composite endpoint of death or heart transplantation (HTx) compared to 68% of patients with GCM (P < 0.001). GCM has a more fulminant clinical course than CS with severe biventricular failure, higher levels of circulating biomarkers and an increased need for HTx. The histopathologic diagnosis remained key determinant even after adjustment for markers of cardiac dysfunction. The online version contains supplementary material available at 10.1186/s12872-022-02639-0.
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影响因子：
3.8
作者：
Bech-Hanssen O;Astengo M;Fredholm M;Bergh N;Hjalmarsson C;Polte CL;Ricksten SE;Bollano E
通讯作者：
Bollano E
DOI：
10.3390/diagnostics12020399
发表时间：
2022-02-03
期刊：
Diagnostics (Basel, Switzerland)
影响因子：
--
作者：
Polte CL;Bobbio E;Bollano E;Bergh N;Polte C;Himmelman J;Lagerstrand KM;Gao SA
通讯作者：
Gao SA
影响因子：
5.4
作者：
Coronado, Michael J.;Bruno, Katelyn A.;Fairweather, DeLisa
通讯作者：
Fairweather, DeLisa
影响因子：
24.3
作者：
Arkema, Elizabeth V.;Grunewald, Johan;Askling, Johan
通讯作者：
Askling, Johan
影响因子：
3.8
作者：
Ekstrom, Kaj;Raisanen-Sokolowski, Anne;Kupari, Markku
通讯作者：
Kupari, Markku