Department of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital (PUMCH), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.
Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Front Immunol. 2022 Aug 2;13:928861. doi: 10.3389/fimmu.2022.928861. eCollection 2022.
Studies concerning myocardial involvement (MI) in patients with anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis/clinically amyopathic dermatomyositis (anti-MDA5 Ab+ DM/CADM) are scarce. We aimed to characterize MI in our anti-MDA5 Ab+ DM/CADM cohort and to investigate its association with prognosis.
In this single-center retrospective study, anti-MDA5 Ab+ hospitalized DM/CADM patients who underwent transthoracic echocardiography (TTE) were enrolled. Myocardial involvement was diagnosed according to abnormal cardiac structure and function detected by TEE. Clinical features and cardiac examination findings of patients with MI were analyzed. Clinical features, laboratory findings, complications, and treatments were compared between MI and non-MI, deceased, and survival patients. Logistic regression analysis was used to explore the independent risk factors for the occurrence of MI and prognostic factors for these patients.
Seventy-six hospitalized patients with anti-MDA5 Ab+ DM/CADM were enrolled. Twelve (15.8%) patients were diagnosed with MI. Of the 12 patients, three underwent cardiac magnetic resonance imaging (CMR) and late gadolinium enhancement (LGE) were noted for them. TEE revealed that eight (66.7%) patients had left atrial and/or ventricular enlargement, three (25.0%) had cardiac hypertrophy, six (50.0%) had diffuse ventricular wall dyskinesia, and seven (58.3%) had diastolic dysfunction. Six (50.0%) patients with MI developed heart failure (HF) during treatment. Of the 12 patients, one patient died of HF caused by myocarditis, three died of infection, and four died of exacerbation of rapidly progressive interstitial lung disease (RP-ILD). Logistic regression analysis revealed that dysphagia (OR 3.923, 95% CI 1.085, 14.181), NT-proBNP >600 pg/ml (OR 18.333, 95% CI 1.508, 222.875), and increased peripheral white blood cells (OR 1.201, 95% CI 1.003, 1.438) were risk factors for the occurrence of MI, but plasma albumin (OR 0.892, 95% CI 0.796, 0.999) was a protective factor. Both MI (OR 5.984, 95% CI 1.174, 30.496) and RP-ILD (OR 11.875, 95% CI 2.796, 50.411) were independent risk factors for the mortality of these anti-MDA5 Ab+ DM/CADM patients.
Myocardial involvement is not rare and is an independent poor prognostic factor of anti-MDA5 Ab+ DM/CADM patients. Cardiac abnormality screening is necessary for them.
抗黑色素瘤分化相关基因 5 抗体阳性皮肌炎/临床无肌病性皮肌炎（抗 MDA5 Ab+DM/CADM）患者心肌受累（MI）的研究较少。我们旨在描述我们的抗 MDA5 Ab+DM/CADM 队列中的 MI，并探讨其与预后的关系。
在这项单中心回顾性研究中，纳入了接受经胸超声心动图（TTE）检查的住院抗 MDA5 Ab+DM/CADM 患者。根据 TEE 检测到的异常心脏结构和功能诊断心肌受累。分析 MI 患者的临床特征和心脏检查结果。比较 MI 与非 MI、死亡和存活患者的临床特征、实验室检查结果、并发症和治疗方法。使用 logistic 回归分析探讨 MI 发生的独立危险因素和这些患者的预后因素。
共纳入 76 例抗 MDA5 Ab+DM/CADM 住院患者。12（15.8%）例患者被诊断为 MI。其中 3 例行心脏磁共振成像（CMR），发现延迟钆增强（LGE）。TEE 显示 8（66.7%）例患者左心房和/或心室扩大，3（25.0%）例患者心脏肥大，6（50.0%）例患者弥漫性室壁运动障碍，7（58.3%）例患者舒张功能障碍。6（50.0%）例 MI 患者在治疗过程中出现心力衰竭（HF）。在 12 例患者中，1 例因心肌炎导致 HF 死亡，3 例因感染死亡，4 例因快速进展性间质性肺病（RP-ILD）加重而死亡。logistic 回归分析显示，吞咽困难（OR 3.923，95%CI 1.085，14.181）、NT-proBNP>600pg/ml（OR 18.333，95%CI 1.508，222.875）和外周白细胞增多（OR 1.201，95%CI 1.003，1.438）是 MI 发生的危险因素，而血浆白蛋白（OR 0.892，95%CI 0.796，0.999）是保护因素。MI（OR 5.984，95%CI 1.174，30.496）和 RP-ILD（OR 11.875，95%CI 2.796，50.411）均是这些抗 MDA5 Ab+DM/CADM 患者死亡的独立危险因素。
心肌受累在抗 MDA5 Ab+DM/CADM 患者中并不少见，是预后不良的独立危险因素。这些患者需要进行心脏异常筛查。
