M.P. Chung, MD, MS, W. Kelly, BS, C.A. Mecoli, MD, MHS, J. Albayda, MD, L. Christopher-Stine, MD, MPH, J.J. Paik, MD, MHS, Division of Rheumatology, Johns Hopkins University School of Medicine;
J. Lovell, MD, N.A. Gilotra, MD, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
J Rheumatol. 2023 Aug;50(8):1039-1046. doi: 10.3899/jrheum.220989. Epub 2023 Apr 1.
To determine the clinical phenotype and outcomes of patients with idiopathic inflammatory myopathies (IIMs) and myocarditis.
Using the Johns Hopkins Myositis Center Research Registry, we identified 31 adult patients with IIM-out of a total of 3082 with confirmed or suspected muscle disease-with an encounter code of myocarditis from 2004 to 2021. Of these, 14 adult patients with IIM were adjudicated to have clinical myocarditis. Information about demographics, autoantibodies, and clinical outcomes was retrospectively collected and analyzed.
Of 14 patients with IIM with clinical myocarditis, the median age at IIM diagnosis was 49 (IQR 35-56) years, and the median age at myocarditis diagnosis was 54 (IQR 36-61) years. The median duration between IIM diagnosis and myocarditis was 3 (IQR 2-9) years. The majority of patients were female (8/14, 57%) and Black (10/14, 71%). Antisynthetase syndrome was the most common IIM subtype (9/14, 64%). Anti-Jo1 (n = 4) and anti-PL12 (n = 3) were the most frequent autoantibodies. At myocarditis diagnosis, most patients (11/14, 79%) had active myositis, defined as elevated creatine kinase and/or muscle weakness; required hospitalization (13/14, 93%); and had reduced left ventricular ejection fraction (LVEF < 50%; 10/14, 71%). Despite intensification of immunosuppression, the 5-year overall survival rate from IIM diagnosis was 84%, and the 5-year overall survival rate from myocarditis diagnosis was 53%. Systolic dysfunction (LVEF < 40%) at final evaluation was observed in all expired patients (n = 6).
Clinical presentations of myocarditis in this select cohort of patients with IIM were severe and heterogeneous with poor outcomes despite intensification of immunosuppression, potentially reflecting late detection of myocarditis.
确定特发性炎性肌病（IIM）和心肌炎患者的临床表型和结局。
使用约翰霍普金斯肌炎中心研究登记处，我们从 2004 年至 2021 年总共 3082 名确诊或疑似肌肉疾病的患者中确定了 31 名成人 IIM 患者，其就诊代码为心肌炎。其中，14 名成人 IIM 患者被判定为患有临床心肌炎。回顾性收集和分析了有关人口统计学、自身抗体和临床结局的信息。
在 14 名患有临床心肌炎的 IIM 患者中，IM 诊断时的中位年龄为 49（IQR 35-56）岁，心肌炎诊断时的中位年龄为 54（IQR 36-61）岁。IM 诊断与心肌炎之间的中位时间为 3（IQR 2-9）年。大多数患者为女性（8/14，57%）和黑人（10/14，71%）。抗合成酶综合征是最常见的 IIM 亚型（9/14，64%）。最常见的自身抗体为抗 Jo1（n = 4）和抗 PL12（n = 3）。在心肌炎诊断时，大多数患者（11/14，79%）患有活动性肌炎，定义为肌酸激酶升高和/或肌肉无力；需要住院治疗（13/14，93%）；和左心室射血分数降低（LVEF <50%；10/14，71%）。尽管免疫抑制作用增强，但从 IIM 诊断到 5 年的总体生存率为 84%，从心肌炎诊断到 5 年的总体生存率为 53%。在所有死亡患者（n = 6）中，最终评估时均观察到收缩功能障碍（LVEF <40%）。
在该特发性炎性肌病患者的选择队列中，心肌炎的临床表现严重且异质，尽管免疫抑制作用增强，但预后仍不佳，这可能反映出心肌炎的晚期发现。
