Classification and Current Management in Juvenile Dermatomyositis

Abstract

Juvenile Dermatomyositis represents 75-80% of juvenile inflammatory myopa- thies. Although it has a low incidence and prevalence, it presents significant mor- bidity due to its cutaneous, muscular, pulmonary, gastrointestinal and cardiac manifestations, among others. It corresponds to a polygenic disorder with multi- ple triggering factors, which determines the development of a vasculopathy that leads to muscle atrophy, inflammation and activation of IFN-1 pathways. Current- ly its diagnosis is based on the EULAR/ACR guidelines (2017). In recent years, dif- ferent subtypes of the disease have been discovered, based on the profile of my- ositis-specific autoantibodies, which has made it possible to establish prognosis and personalized therapeutic strategies. Pharmacological management continues to be based mainly on the use of corticosteroids and DMARDs, as well as biological therapy; In recent years, JAK inhibitors have shown promising results, becoming the newest therapeutic alternative for disease control.