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伴有心内膜血栓导致的心室中部梗阻这一不寻常表现的嗜酸性肉芽肿性多血管炎(EGPA)。

Eosinophilic Granulomatosis with Polyangiitis (EGPA) with an Unusual Manifestation of Mid-Ventricular Obstruction Caused by Endocardial Thrombus.

作者信息

Ito Takahide, Fujita Shu-Ichi, Kanzaki Yumiko, Sohmiya Koichi, Hoshiga Masaaki

机构信息

Department of Cardiology, Osaka Medical College, Takatsuki, Osaka, Japan.

出版信息

Am J Case Rep. 2018 Oct 8;19:1197-1203. doi: 10.12659/AJCR.910861.

DOI:10.12659/AJCR.910861
PMID:30293983
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6187986/
Abstract

BACKGROUND Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis of unknown cause accompanied by prominent eosinophilia. Intracardiac thrombosis is one of the major cardiac complications in EGPA that may cause thromboembolism. CASE REPORT A 46-year-old male presenting with intermittent chest pain and numbness of the lower extremities was admitted to our center. His case was complicated by multiple brain infarcts and endocardial thrombosis in the left ventricle. A condition of restrictive cardiomyopathy was also found. After a thorough workup, he was diagnosed with antineutrophil cytoplasmic antibody (ANCA) positive EGPA. Interestingly, the thrombus was accompanied by a pressure gradient producing mid-ventricular obstruction. The patient improved reasonably with immunosuppression and anticoagulation treatment, in addition to heart failure treatment, and had a concomitant regression of the thrombus and reduction of the pressure gradient. CONCLUSIONS For an EGPA patient complicated by intraventricular obstruction caused by endocardial thrombosis, which could accelerate the release of the thrombus into the systemic circulation resulting in a life-threating condition, timely and aggressive measures against cardioembolic complications should be considered.

摘要

背景

嗜酸性肉芽肿性多血管炎(EGPA)是一种病因不明的罕见系统性血管炎,伴有显著的嗜酸性粒细胞增多。心内血栓形成是EGPA的主要心脏并发症之一,可能导致血栓栓塞。病例报告:一名46岁男性因间歇性胸痛和下肢麻木入住我院。他的病例并发多发性脑梗死和左心室内膜血栓形成。还发现了限制性心肌病。经过全面检查,他被诊断为抗中性粒细胞胞浆抗体(ANCA)阳性的EGPA。有趣的是,血栓伴有产生心室中部梗阻的压力梯度。除心力衰竭治疗外,患者经免疫抑制和抗凝治疗后病情合理改善,血栓随之消退,压力梯度降低。结论:对于并发心内膜血栓形成导致心室梗阻的EGPA患者,心内膜血栓形成可加速血栓释放至体循环,从而导致危及生命的状况,应考虑及时采取积极措施预防心脏栓塞并发症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7816/6187986/4dbb592ad6ee/amjcaserep-19-1197-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7816/6187986/e26aaf0294a7/amjcaserep-19-1197-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7816/6187986/16bac63cdd61/amjcaserep-19-1197-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7816/6187986/c10e2ad56845/amjcaserep-19-1197-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7816/6187986/15e648c00e18/amjcaserep-19-1197-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7816/6187986/4dbb592ad6ee/amjcaserep-19-1197-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7816/6187986/e26aaf0294a7/amjcaserep-19-1197-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7816/6187986/16bac63cdd61/amjcaserep-19-1197-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7816/6187986/c10e2ad56845/amjcaserep-19-1197-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7816/6187986/15e648c00e18/amjcaserep-19-1197-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7816/6187986/4dbb592ad6ee/amjcaserep-19-1197-g005.jpg

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ANCA-Negative Churg-Strauss Syndrome Presenting as Acute Multiple Cerebral Infarcts: A Case Report.以急性多发性脑梗死为表现的抗中性粒细胞胞浆抗体阴性的变应性肉芽肿性血管炎:一例报告
J Stroke Cerebrovasc Dis. 2017 Mar;26(3):e47-e49. doi: 10.1016/j.jstrokecerebrovasdis.2016.12.009. Epub 2017 Jan 5.
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Multiple Thromboembolic Cerebral Infarctions from the Aorta in a Patient with Churg-Strauss Syndrome.
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