一例狼疮性冠状动脉血管炎致缺血性心肌病心脏移植的病例报告。

A case report of heart transplant for ischaemic cardiomyopathy from lupus coronary vasculitis.

作者信息

Nandkeolyar Shuktika, Kim Hyungjin B, Doctorian Tanya, Stoletniy Liset N, Sandhu Vaneet K, Yu Micah, Zuppan Craig W, Razzouk Anees, Hilliard Anthony, Parwani Purvi

机构信息

Division of Cardiology, Department of Medicine, Loma Linda University Medical Center, 11234 Anderson St. Room 4404, Loma Linda, CA 92354, USA.

Division of Rheumatology, Loma Linda University Medical Center, 11234 Anderson St. Room 3650, Loma Linda, CA 92354, USA.

出版信息

Eur Heart J Case Rep. 2019 Oct 31;3(4):1-7. doi: 10.1093/ehjcr/ytz183. eCollection 2019 Dec.

DOI:10.1093/ehjcr/ytz183

PMID:32123802

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7042145/

Abstract

BACKGROUND

Coronary vasculitis is a rare, life-threatening complication of systemic lupus erythematosus (SLE).

CASE SUMMARY

A 23-year-old woman with SLE presented with typical angina and worsening dyspnoea on exertion. Coronary angiography revealed severe triple vessel disease with a 'string of beads' appearance classic for coronary vasculitis. Transthoracic echocardiogram revealed ejection fraction of 25-30% with a severely hypokinetic distal septum and distal anterior wall and an akinetic apical wall. Despite vasculitis treatment with cyclophosphamide and pulse-dose steroids, her coronary vasculitis did not improve. She was refractory to anti-anginal and guideline-directed medical therapy for heart failure and successfully underwent orthotopic heart transplant (OHT).

DISCUSSION

This is the first reported case of OHT in the case of SLE coronary vasculitis. Chronic SLE coronary vasculitis is caused by lymphocyic infiltration leading to inflammation and fibrosis of the major epicardial coronary arteries but can be successfully managed with OHT when refractory to medical SLE and heart failure therapies. It can affect patients of all ages with SLE, emphasizing the importance of thorough history taking and clinical evaluation in young patients presenting with cardiac symptoms to establish an appropriate diagnosis and treatment plan.

摘要

背景

冠状动脉血管炎是系统性红斑狼疮（SLE）一种罕见的、危及生命的并发症。

病例摘要

一名23岁的SLE女性患者出现典型心绞痛，且运动时呼吸困难加重。冠状动脉造影显示严重的三支血管病变，呈现出冠状动脉血管炎典型的“串珠样”外观。经胸超声心动图显示射血分数为25%-30%，室间隔远端、前壁远端严重运动减弱，心尖壁运动消失。尽管使用环磷酰胺和冲击剂量类固醇进行血管炎治疗，但其冠状动脉血管炎并未改善。她对抗心绞痛及针对心力衰竭的指南指导药物治疗无效，最终成功接受了原位心脏移植（OHT）。

讨论

这是首例报道的SLE冠状动脉血管炎患者接受OHT的病例。慢性SLE冠状动脉血管炎是由淋巴细胞浸润导致主要心外膜冠状动脉炎症和纤维化引起的，但当对SLE及心力衰竭治疗无效时，OHT可成功治疗该疾病。它可影响所有年龄段的SLE患者，这凸显了对出现心脏症状的年轻患者进行全面病史采集和临床评估以确立恰当诊断和治疗方案的重要性。