目的:本研究旨在通过聚类分析将特发性炎症性肌病(IIM)合并心脏受累(IIM-CI)患者根据其临床表型分为不同类别,并探讨其预后差异。
方法:检索2015年1月至2021年6月北京协和医院收治的IIM-CI患者。临床数据,实验室检查,和治疗进行回顾性审查,结果被追踪了.采用二阶聚类方法进行分类。
结果:本研究共纳入88例IIM-CI患者,通过聚类分析将其分为两类。第一类包括表现出明显心脏结构和功能变化的患者,如心房和/或心室扩大,以及显著的心功能不全生物标志物,而II类患者表现出更广泛的全身性损伤和严重的骨骼肌无力。相比之下,肺动脉高压(58.8%vs16.7%,p<0.01),心律失常(82.4%vs27.8%,p<0.01),血清抗线粒体M2抗体阳性(52.9%vs5.6%,p<0.01)在第一类中比在第二类中更普遍,和血清N末端B型利钠肽水平(1703.5pg/Lvs364.0pg/L,p=0.02)在I类中显着升高,而骨骼肌无力(50.0%vs74.1%,p=0.02),间质性肺病(20.6%vs63.0%,p<0.01),皮疹(11.8%vs48.1%,p<0.01),关节痛(2.9%vs27.8%,p<0.01),发烧(2.9%vs27.8%,p<0.01),和吞咽困难(2.9%vs22.2%,p<0.01)在II类患者中更为常见。心力衰竭是第一类死亡的主要原因,但严重肺炎是II类死亡的主要原因.
结论:根据具有独特特征的临床特征确定了两类IIM-CI。两类患者的临床表现存在差异,自身抗体谱,和死亡的主要原因。
OBJECTIVE: This study aimed to classify idiopathic inflammatory myopathy (IIM) patients with cardiac involvement (IIM-CI) into different categories based on their clinical phenotypes via cluster analysis and to explore their differences in outcomes.
METHODS: IIM-CI patients admitted to Peking Union Medical College Hospital from January 2015 to June 2021 were retrieved. The clinical data, laboratory examinations, and treatment were retrospectively reviewed, and the outcome was traced. A second-order clustering method was employed for categorization.
RESULTS: A total of 88 IIM-CI patients were enrolled in this study and were classified into two categories through cluster analysis. Category I consisted of patients who exhibited distinct cardiac structural and functional changes, such as enlargement of atriums and/or ventricles, along with the remarkable heart insufficiency biomarkers, whereas patients of category II displayed more widely systemic injuries and intensive skeletal muscle weakness. In comparison, pulmonary hypertension (58.8% vs 16.7%, p < 0.01), arrhythmia (82.4% vs 27.8%, p < 0.01), and positive serum anti-mitochondrial-M2 antibody (52.9% vs 5.6%, p < 0.01) were more prevalent in category I than in category II, and serum N-terminal pro-B-type natriuretic peptide levels (1703.5 pg/L vs 364.0 pg/L, p = 0.02) were significantly elevated in category I, whereas skeletal muscle weakness (50.0% vs 74.1%, p = 0.02), interstitial lung disease (20.6% vs 63.0%, p < 0.01), skin rash (11.8% vs 48.1%, p < 0.01), arthralgia (2.9% vs 27.8%, p < 0.01), fever (2.9% vs 27.8%, p < 0.01), and dysphagia (2.9% vs 22.2%, p < 0.01) were more common in category II patients. Heart failure was the primary cause of death in category I, but severe pneumonia was predominantly responsible for deaths in category II.
CONCLUSIONS: Two categories of IIM-CI were identified based on clinical features with distinctive characteristics. Two categories exhibited differences in clinical manifestations, autoantibody profiles, and the primary cause of death.