Cardiac involvement in adult and juvenile idiopathic inflammatory myopathies

Abstract

Idiopathic inflammatory myopathies (IIM) include the main subgroups polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM) and juvenile DM (JDM). The mentioned subgroups are characterised by inflammation of skeletal muscles leading to muscle weakness and other organs can also be affected as well. Even though clinically significant heart involvement is uncommon, heart disease is one of the major causes of death in IIM. Recent studies show an increased prevalence of traditional cardiovascular risk factors in JDM and DM/PM, which need attention. The risk of developing atherosclerotic coronary artery disease is increased twofold to fourfold in DM/PM. New and improved diagnostic methods have in recent studies in PM/DM and JDM demonstrated a high prevalence of subclinical cardiac involvement, especially diastolic dysfunction. Interactions between proinflammatory cytokines and traditional risk factors might contribute to the pathogenesis of cardiac dysfunction. Heart involvement could also be related to myocarditis and/or myocardial fibrosis, leading to arrhythmias and congestive heart failure, demonstrated both in adult and juvenile IIM. Also, reduced heart rate variability (a known risk factor for cardiac morbidity and mortality) has been shown in long-standing JDM. Until more information is available, patients with IIM should follow the same recommendations for cardiovascular risk stratification and prevention as for the corresponding general population, but be aware that statins might worsen muscle symptoms mimicking myositis relapse. On the basis of recent studies, we recommend a low threshold for cardiac workup and follow-up in patients with IIM.

Multiparametric cardiovascular magnetic resonance characteristics and dynamic changes in myocardial and skeletal muscles in idiopathic inflammatory cardiomyopathy

“…The typical clinical manifestations of IIM include proximal muscle weakness, myalgia, and dyspnea related to respiratory muscle dysfunction due to systemic impairment of skeletal muscles. Myocardial involvement could be subclinical, but it is a major cause of adverse outcomes in patients with IIM [1][2][3][4]. Clinical manifestations of cardiac involvement in IIM patients can range from none to arrhythmia, heart failure, and even cardiac death [5].…”

Early detection of myocardial involvement by T1 mapping of cardiac MRI in idiopathic inflammatory myopathy

“…Subclinical cardiac involvement is reported in 13–72% of patients, including nonspecific electrocardiogram (ECG) and echocardiogram abnormalities . Moreover, the reported mortality due to cardiac involvement in adult onset IIM varies from 14–55%, even in subclinical types. Although cardiac involvement is not rare in IIM, early detection of subclinical myocardial abnormalities that may lead to the development of preventive intervention remains challenging.…”

Does Pulmonary Involvement Play a Role in Right Ventricular Dysfunction of Patients with Idiopathic Inflammatory Myopathies?

“…In addition to patients with ILD, subclinical cardiac dysfunction has been detected in populations with diabetes, dyslipidemia, and hypertension 4,5,6 , and these traditional CV risk factors are more prevalent in patients with IIM than in the general population 7 , so we cannot deny that reduced myocardial strain in patients with IIM is the interaction between comorbidities and inflammation/fibrosis in the myocardium, due to the disease per se. But speckle echocardiography still remains as the most effective screening method for cardiac dysfunction.…”