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一名新诊断为Churg-Strauss综合征(CSS)患者的Loeffler心内膜炎:病例报告

Loeffler's endocarditis in a patient with a new diagnosed Churg-Strauss syndrome (CSS): A case report.

作者信息

Lin Wei-Chen, Huang Kaun-Chih, Hsiung Ming-Chen, Feng An-Ning

机构信息

Department of Cardiology, Cheng Hsin General Hospital, Taipei, Taiwan.

出版信息

Caspian J Intern Med. 2021 Winter;12(1):107-110. doi: 10.22088/cjim.12.1.107.

DOI:10.22088/cjim.12.1.107
PMID:33680407
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7919172/
Abstract

BACKGROUND

Loeffler's endocarditis is a rare disease, caused by endocardial involvement of esosinophils, which damages the heart and leads to endomyocardial fibrosis with consequent restrictive cardiomyopathy, mural thrombi or valvular dysfunction. The association between Loeffler's endocarditis and Churg-Strauss syndrome (CSS) was also reported. Abnormal elevation of peripheral eosinophil counts in a heart failure patient is a hint of disease. Though echocardiography or cardiac magnetic resonance imaging (MRI) facilitates diagnosis, endomyocardial biopsy is still the gold standard. Treatments include immunosuppressive agents, anticoagulant and guideline-directed medical therapy for heart failure.

CASE PRESENTATION

A 59-year-old man presented with progressive dyspnea for one week and he was referred to our hospital for surgical treatment evaluation because valve destruction by infective endocarditis has been suspected at the local hospital. Echocardiography revealed biventricular mural thrombus and limited aortic valve opening caused by abutting thrombus. Moreover, eosinophilia, bronchial asthma, lung infiltration, acute kidney injury and positive perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) test implied Churg Strauss syndrome. Eosinophil infiltrate with fibrin thrombus was revealed by endomyocardial biopsy. The patient was diagnosed of cardiac involvement of CSS and recovered after immunosuppressive and anticoagulant treatments.

CONCLUSION

Loeffler's endocarditis should be suspected when physicians encounter restrictive cardiomyopathy accompanied by mural thrombus in a patient with eosinophilia. Prompt immunosuppressive and anticoagulant medication can bring the disease under control.

摘要

背景

吕弗勒心内膜炎是一种罕见疾病,由嗜酸性粒细胞累及心内膜引起,可损害心脏并导致心内膜心肌纤维化,进而引发限制性心肌病、壁血栓形成或瓣膜功能障碍。吕弗勒心内膜炎与变应性肉芽肿性血管炎(CSS)之间的关联也有报道。心力衰竭患者外周嗜酸性粒细胞计数异常升高是该病的一个提示。尽管超声心动图或心脏磁共振成像(MRI)有助于诊断,但心内膜心肌活检仍是金标准。治疗方法包括免疫抑制剂、抗凝剂以及针对心力衰竭的指南导向药物治疗。

病例介绍

一名59岁男性因进行性呼吸困难1周就诊,因其在当地医院被怀疑患有感染性心内膜炎导致瓣膜破坏,故而转诊至我院进行手术治疗评估。超声心动图显示双心室壁血栓以及由毗邻血栓导致的主动脉瓣开放受限。此外,嗜酸性粒细胞增多、支气管哮喘、肺部浸润、急性肾损伤以及核周型抗中性粒细胞胞浆抗体(p-ANCA)检测阳性提示变应性肉芽肿性血管炎。心内膜心肌活检显示嗜酸性粒细胞浸润伴纤维蛋白血栓形成。该患者被诊断为变应性肉芽肿性血管炎累及心脏,经免疫抑制和抗凝治疗后康复。

结论

当医生遇到嗜酸性粒细胞增多的患者伴有限制性心肌病及壁血栓时,应怀疑吕弗勒心内膜炎。及时给予免疫抑制和抗凝药物治疗可控制病情。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d98d/7919172/2cd91ba42df2/cjim-12-107-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d98d/7919172/682dade2bc24/cjim-12-107-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d98d/7919172/2cd91ba42df2/cjim-12-107-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d98d/7919172/682dade2bc24/cjim-12-107-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d98d/7919172/2cd91ba42df2/cjim-12-107-g002.jpg

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Hypereosinophilic syndrome: cardiac diagnosis and management.嗜酸性粒细胞增多综合征:心脏诊断与管理。
Heart. 2016 Jan;102(2):100-6. doi: 10.1136/heartjnl-2015-307959. Epub 2015 Nov 13.
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Churg-Strauss syndrome.变应性肉芽肿性血管炎。
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