Cardiac involvement in pediatric systemic autoimmune diseases has a wide spectrum of
presentation ranging from asymptomatic to severe clinically overt involvement. Coronary artery
disease, pericardial, myocardial, valvular and rythm disturbances are the most common causes of heart
lesion in pediatric systemic autoimmune diseases and cannot be explained only by the traditional
cardiovascular risk factors. Therefore, chronic inflammation has been considered as an additive
causative factor of cardiac disease in these patients.
Rheumatic fever, juvenile idiopathic arthritis, systemic lupus erythematosus, ankylosing spondylitis/spondyloarthritides,
juvenile scleroderma, juvenile dermatomyositis/polymyositis, Kawasaki disease and other autoimmune vasculitides are
the commonest pediatric systemic autoimmune diseases with heart involvement.
Noninvasive cardiovascular imaging is an absolutely necessary adjunct to the clinical evaluation of these patients.
Echocardiography is the cornerstone of this assessment, due to excellent acoustic window in children, lack of radiation,
low cost and high availability. However, it can not detect disease acuity and pathophysiologic background of cardiac
lesions. Recently, the development of cardiovascular magnetic resonance imaging holds the promise for early detection of
subclinical heart disease and detailed serial evaluation of myocardium (function, inflammation, stress perfusion-fibrosis)
and coronary arteries (assessment of ectasia and aneurysms).