系统性硬化症合并心肌受累患者的临床特征和结局。
The clinical characteristics and outcomes of patients with systemic sclerosis with myocardial involvement.
机构信息
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital (PUMCH), Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology; State Key Laboratory of Complex Severe and Rare Diseases; Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, No.1 Shuaifuyuan Wangfujing Dongcheng District, Beijing, 100730, China.
Department of Cardiology, Peking Union Medical College Hospital, Beijing, China.
出版信息
Orphanet J Rare Dis. 2023 Apr 14;18(1):83. doi: 10.1186/s13023-023-02699-1.
BACKGROUND
Myocardial involvement (MI) is the primary cause of death in patients with systemic sclerosis (SSc). We analyzed patients with SSc and MI to identify their characteristics and outcome.
METHOD
We retrospectively collected data from SSc patients with MI admitted to Peking Union Medical College Hospital between January 2012 and May 2021. SSc patients without MI were randomly selected as controls after matching age and gender at a ratio of 1:3.
RESULTS
In total, 21 SSc patients (17 females) with MI were enrolled. The mean age at SSc onset was 42.3 ± 15.1 years old. Compared with controls, myositis (42.9% vs. 14.3%, P = 0.014) and elevation of CK (33.3% vs. 4.8%, P = 0.002) were more common in patients with MI. Of the 7 patients without cardiovascular symptoms, 3 /5 showed elevations in cardiac troponin-I (cTnI), 6 showed elevations of N-terminal brain natriuretic peptide (NT-proBNP). Eleven patients were followed up for a median period of 15.5 months and four patients developed newly occurring left ventricular ejection fraction (LVEF) < 50%.
CONCLUSION
One third of SSc patients with MI were asymptomatic. Regular monitoring of CTnI, NT-proBNP and echocardiography is helpful for the diagnosis of MI during the early stages. Its prognosis is poor.
背景
心肌受累（MI）是系统性硬化症（SSc）患者死亡的主要原因。我们分析了合并 MI 的 SSc 患者，以明确其特征和结局。
方法
我们回顾性收集了 2012 年 1 月至 2021 年 5 月期间在北京协和医院住院的 SSc 合并 MI 患者的数据。按照 1:3 的比例，根据年龄和性别匹配选择无 MI 的 SSc 患者作为对照。
结果
共纳入 21 例（17 例女性）合并 MI 的 SSc 患者。SSc 发病时的平均年龄为 42.3±15.1 岁。与对照组相比，肌炎（42.9%比 14.3%，P=0.014）和 CK 升高（33.3%比 4.8%，P=0.002）在 MI 患者中更为常见。在 7 例无心血管症状的患者中，5/7 例患者的心脏肌钙蛋白 I（cTnI）升高，6/7 例患者的 N 末端脑钠肽前体（NT-proBNP）升高。11 例患者的中位随访时间为 15.5 个月，4 例患者新发左心室射血分数（LVEF）<50%。
结论
三分之一的合并 MI 的 SSc 患者无症状。定期监测 cTnI、NT-proBNP 和超声心动图有助于早期诊断 MI。其预后不良。
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