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一例以Libman-Sacks心内膜炎为表现的系统性红斑狼疮患者

A Case of Systemic Lupus Erythematosus in a Patient Presenting with Libman-Sacks Endocarditis.

作者信息

Al-Jehani Mariann, Al-Husayni Faisal, Almaqati Ahmed, Shahbaz Jomanah, Albugami Saad, Alameen Wail

机构信息

King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia.

King Abdullah International Medical Research Center, Jeddah, Saudi Arabia.

出版信息

Case Rep Cardiol. 2021 Aug 31;2021:5573141. doi: 10.1155/2021/5573141. eCollection 2021.

DOI:10.1155/2021/5573141
PMID:34513093
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8424240/
Abstract

BACKGROUND

Libman-Sacks endocarditis (LSE) is a rare cardiovascular manifestation of systemic lupus erythematosus/antiphospholipid syndrome that is described as a sterile verrucous nonbacterial vegetative lesion. These lesions can cause progressive damage to the heart valves leading to valve surgery. The most common valves to be affected are the aortic and mitral valves. Libman-Sacks endocarditis is associated with malignancies, other systemic diseases like systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APS). The majority of LSE patients are usually asymptomatic. . We describe a 39-year-old male patient who presented with increasing shortness of breath and pulmonary congestion. He was found to have severe mitral valve regurgitation and mitral stenosis. Transesophageal echocardiogram confirmed the diagnosis of Libman-Sacks endocarditis with thickened mitral valve leaflets with symmetrical mass-like structure causing a restriction in the valve function during both cardiac phases later diagnosed with systemic lupus erythematosus by immunology. The patient was started on diuretics, anticoagulants, angiotensin inhibitors, beta-blockers, and hydroxychloroquine. He underwent successful mechanical mitral valve replacement with a 27 mm St. Jude valve. The mitral valve was found to be grossly thickened with friable tissue and complete amalgamation of the leaflets with subvalvular apparatus. The patient suffered some warfarin adverse effects a year later but did well otherwise.

CONCLUSION

This case demonstrates that Libman-Sacks endocarditis can be the first manifestation of systemic lupus erythematosus. Early and prompt diagnosis of LSE can prevent and lessen the many side effects associated with thromboembolism. Additionally, addressing the underlying disease is key to successful treatment.

摘要

背景

利布曼-萨克斯心内膜炎(LSE)是系统性红斑狼疮/抗磷脂综合征罕见的心血管表现,被描述为无菌性疣状非细菌性赘生物病变。这些病变可导致心脏瓣膜进行性损害,进而需要进行瓣膜手术。最常受累的瓣膜是主动脉瓣和二尖瓣。利布曼-萨克斯心内膜炎与恶性肿瘤、系统性红斑狼疮(SLE)和抗磷脂抗体综合征(APS)等其他系统性疾病有关。大多数LSE患者通常无症状。我们描述了一名39岁男性患者,他出现呼吸急促和肺充血加重。发现他患有严重的二尖瓣反流和二尖瓣狭窄。经食管超声心动图确诊为利布曼-萨克斯心内膜炎,二尖瓣叶增厚,有对称的团块状结构,在两个心动周期均导致瓣膜功能受限,后来经免疫学诊断为系统性红斑狼疮。患者开始使用利尿剂、抗凝剂、血管紧张素抑制剂、β受体阻滞剂和羟氯喹。他成功接受了27毫米圣犹达瓣膜的机械二尖瓣置换术。发现二尖瓣明显增厚,组织易碎,瓣叶与瓣下装置完全融合。患者一年后出现一些华法林不良反应,但其他方面情况良好。

结论

本病例表明利布曼-萨克斯心内膜炎可能是系统性红斑狼疮的首发表现。早期及时诊断LSE可预防和减轻与血栓栓塞相关的许多副作用。此外,治疗潜在疾病是成功治疗的关键。

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