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

Abstract

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.

Physiotherapy in Juvenile Dermatomyositis

“…Epidemiology JDM is a rare autoimmune disease that affects all nations, with a prevalence of 2.5-4.1 children per million in the United States 5 . The average age of onset is approximately 7 years and commonly leads a chronic course 6,8 .…”

“…The average age of onset is approximately 7 years and commonly leads a chronic course 6,8 . JDM accounts for more than 80% of the idiopathic inflammatory myopathy childhood cases, thus making it the most common idiopathic inflammatory myopathy among children with a 5:1 ratio of female to male 5,9 .…”

Juvenile dermatomyositis: new insights and new treatment strategies

“…The Cutaneous Assessment Tool (CAT) assesses skin disease in myositis by considering activity in 10 lesions, damage in four lesions and a combination of activity and damage in seven lesions. The tool has been partially validated in a large JDM population and demonstrated good reliability, content and construct validity, and responsiveness [Huber and Feldman, 2005;Huber et al 2008aHuber et al , 2008b. Assessment of other organ systems, including respiratory, gastrointestinal, speech and language is also critical.…”

The treatment and prognosis of dermatomyositis: An updated review

“…153 One article 153 noted that before corticosteroid therapy, death rates in JDM were near 34%. Today, these are much lower, as newer studies found mortality at 1.5% 39 and even 0%.…”