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经形态学证实的抗中性粒细胞胞浆抗体阳性的吕弗勒全心炎:内科及外科治疗

Morphologically proved ANCA positive Loeffler's pancarditis: medical and surgical treatment.

作者信息

Blagova O V, Aliyeva I N, Nedostup A V, Kogan E A, Komarov R N, Chernyavsky S V, Seslavinskaya V V, Shelukha P A, Sedov V P, Gagarina N V, Mershina E A, Sinitsyn V E, Fomin V V

机构信息

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), Moscow, Russia.

Federal Center of Treatment and Rehabilitation of the Ministry of Health of the Russian Federation, Moscow, Russia.

出版信息

Ter Arkh. 2019 May 16;91(4):99-106. doi: 10.26442/00403660.2019.04.000048.

Abstract

Loeffler's endocarditis remains is a very rare disease, develops due to eosinophilic inflammation predominantly of the endocardium with an outcome in fibrosis and massive thrombus formation and. He is generally characterized by an unfavorable prognosis. Clinical case of a 42-year-old patient with Loeffler endocarditis is presented. The development of the disease was preceded by a polyvalent allergy, mild dry eye syndrome and pansinusitis with a single eosinophilia of blood up to 16%. The reason for the hospitalization was the appearance of biventricular heart failure. During the previous year, the level of blood eosinophils remained normal, a threefold increase in the level of eosinophilic cationic protein was observed once. A 20-fold increase in the pANCA level, a 2.5-fold increase in the level of antibodies to DNA, an antibody to the nuclei of cardiomyocytes 1:160 were detected. The diagnosis was made on the basis of electrocardiography data (low QRS voltage, atrial hypertrophy), echocardiography, multispiral computed tomography and magnetic resonance imaging of the heart (thickening and delayed contrasting of the endocardium, massive thrombosis of the left ventricular apex with obliteration of its cavity, encapsulated fluid in the pericardium with compression of the right ventricle). Systolic dysfunction, severe signs of restriction and arrhythmias were absent. Trombectomy, tricuspid valve plasty, pericardial resection, suturing of an open oval window were performed. Signs of active inflammation with single eosinophils, vasculitis, perimuscular sclerosis, endocardial sclerosis were detected in morphological and immunohistochemical studies of endo-, myo-, pericardium. Viral genome was not found. The therapy with methylprednisolone 24 mg/day, azathioprine 75 mg/day was started. Six months after the operation, the symptoms of heart failure are completely absent, the thrombosis did not recur.

摘要

吕弗勒心内膜炎仍然是一种非常罕见的疾病,主要由于心内膜的嗜酸性炎症发展而来,最终导致纤维化和大量血栓形成。其通常预后不良。本文介绍了一名42岁吕弗勒心内膜炎患者的临床病例。该疾病发生前有多种过敏反应、轻度干眼症综合征和全鼻窦炎,血液中嗜酸性粒细胞单次升高至16%。住院原因是出现双心室心力衰竭。在前一年,血液嗜酸性粒细胞水平保持正常,嗜酸性阳离子蛋白水平曾有一次三倍升高。检测到pANCA水平升高20倍,抗DNA抗体水平升高2.5倍,抗心肌细胞核抗体1:160。诊断基于心电图数据(低QRS电压、心房肥大)、超声心动图、心脏多排螺旋计算机断层扫描和磁共振成像(心内膜增厚和延迟强化、左心室心尖大量血栓形成伴腔隙闭塞、心包内包裹性积液伴右心室受压)。无收缩功能障碍、严重的限制征象和心律失常。进行了血栓切除术、三尖瓣成形术、心包切除术、开放卵圆孔缝合术。在心内膜、心肌、心包的形态学和免疫组织化学研究中检测到有单个嗜酸性粒细胞的活动性炎症迹象、血管炎、肌周硬化、心内膜硬化。未发现病毒基因组。开始使用甲泼尼龙24毫克/天、硫唑嘌呤75毫克/天进行治疗。术后六个月,心力衰竭症状完全消失,血栓未复发。

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