RESUMEN
In addition to skin and muscle lesions,severe juvenile dermatomyositis can also cause multiple systemic and organ damage,which directly threatens the life of children.The development of imaging and specific antibody detection techniques is beneficial to the early diagnosis of severe juvenile dermatomyositis.Early diagnosis and regular combination of continuous medication can improve its prognosis.At present,the treatment of severe juvenile dermatomyositis is mainly based on large doses of glucocorticoid.Intravenous immunoglobulin is a rapid and effective treatment and can be used as a first-line drug.It can be repeated in half a year.For children with combined organ damage,selective use of cyclosporine,mycophenolate mofetil,tacrolimus and other immunosuppressive agents,even biological agents,plasma exchange,stem cell transplantation,etc.,but the efficacy needs to be further verified.