Hongji Zhu,1,2,* Runzhao Li,1,2,* Hongxia Tan,1,* Tangdan Ding,1,2 Ying Yuan,3 Zhihua Wen,4 Jijun Zhao,5 Min Liu,1 Qiong Shi,1 Liubing Li1 

1Department of Laboratory Medicine, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, 510080, People’s Republic of China; 2Department of Laboratory Medicine, Nansha Division of the First Affiliated Hospital, Sun Yat-sen University, Guangzhou, 510080, People’s Republic of China; 3Department of Cardiology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, 510080, People’s Republic of China; 4Department of Radiology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong, 510080, People’s Republic of China; 5Department of Rheumatology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, 510080, People’s Republic of China

*These authors contributed equally to this work

Correspondence: Liubing Li, Department of Laboratory Medicine, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, 510080, People’s Republic of China, Email lilb8@mail.sysu.edu.cn Qiong Shi, Department of Laboratory Medicine, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, 510080, People’s Republic of China, Email sq164258@163.com

Abstract: Idiopathic inflammatory myopathies (IIMs) are a group of autoimmune diseases that includes the main subtypes dermatomyositis, polymyositis, immune-mediated necrotizing myopathy, and inclusion body myositis. IIMs are characterized by the involvement of skeletal muscle and multiple organs, including the heart. This review summarizes the pathology, prevalence, biomarkers, imaging and treatment of cardiac involvement in patients with IIMs. The cardiac involvement in these patients is usually subclinical and rarely considered as the main clinical feature at the time of initial consultation, with a prevalence ranging from 4% to 26%. However, it results in a worse prognosis and represents the main cause of mortality in patients with IIMs. The selection of specific serum cardiac biomarkers is essential for the early detection of cardiac involvement in patients with IIMs, such as cardiac troponin I (cTnI), which is preferred over cardiac troponin T (cTnT), followed by diagnostic evaluations including electrocardiography (ECG), echocardiography (ECHO), and cardiac magnetic resonance imaging (CMR). The combination of glucocorticoids, immunosuppressants, and conventional cardiac medications is effective for the management of IIM patients with confirmed cardiac involvement.

Keywords: IIMs, cardiac involvement, prevalence, biomarkers, imaging, treatment