Clinical Characteristics and Outcomes of Juvenile and Adult Dermatomyositis

Dermatomyositis (DM) is an idiopathic inflammatory myopathy with bimodal onset age distribution. The age of onset is between 5-18 yr in juvenile DM and 45-64 yr in adult DM. DM has a distinct clinical manifestation characterized by proximal muscle weakness, skin rash, extramuscular manifestations (joint contracture, dysphagia, cardiac disturbances, pulmonary symptoms, subcutaneous calcifications), and associated disorders (connective tissue disease, systemic autoimmune diseases, malignancy). The pathogenesis of juvenile and adult DM is presumably similar but there are important differences in some of the clinical manifestations, associated disorders, and outcomes. In this study, we investigated the clinical characteristics and outcomes of 16 patients with juvenile DM and 48 with adult DM. This study recognizes distinctive characteristics of juvenile DM such as higher frequency of neck muscle involvement, subcutaneous calcifications, and better outcomes

Safety and efficacy of exercise training in patients with an idiopathic inflammatory myopathy—a systematic review

Objective. Idiopathic inflammatory myopathies (IIMs) are a group of rare heterogeneous autoimmune skeletal muscle disorders characterized by muscle weakness, excessive muscle fatigue and diminished aerobic fitness. Exercise training could be one way to prevent or delay the negative effects of the disease and the impairments seen in patients with an IIM. The objective was to examine whether exercise training is safe and effective in patients with an IIM. Methods. All experimental studies that assessed the safety and/or efficacy of an exercise training programme in patients with an IIM except for case studies were reviewed. Pre-MEDLINE, MEDLINE and EMBASE database searching was done up to November 2010. Information was extracted on the number of participants, characteristics of participants, type of intervention, type of outcome measure, type of study design, report characteristics, geographical origin and risk of bias within studies. The change (percentage and significance) in group mean or median for each outcome measure in each study was determined as well. Results. Two randomized controlled trials, one non-randomized controlled trial and nine uncontrolled trials were included. No studies in children were found. Safety measures did not worsen and efficacy measures improved or did not change. Most of the included studies had a high selection and/or allocation bias. Conclusions. In conclusion, it appears that exercise training is safe and effective in adult patients with active as well as inactive stable IIMs. However, more studies with a well-controlled design are needed. In addition, studies in children with an IIM are indicated