Long-term outcomes in juvenile dermatomyositis: how did we get here and where are we going?

Juvenile dermatomyositis (JDM) affects two to four children per million. Prior to treatment with corticosteroids, JDM had a high mortality rate (> 30%) and left 50% of those who survived with serious permanent impairments. After the introduction of corticosteroids, mortality rapidly dropped to less than 10%, and is currently reported to be less than 2% to 3%. Because most children now survive this illness, there is greater interest in long term outcomes. However, review of the literature shows while much is known about mortality, relatively little is known about long term outcomes such as physical function, quality of life, pain, educational and vocational achievement, patient satisfaction, and ongoing disease activity. Furthermore, the literature that has been published has not typically used the same outcomes making comparisons across studies difficult. Current efforts to identify key outcomes and validate measures for those outcomes will allow researchers in the future to provide this much needed information.