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Myositis specific autoantibodies (MSA), exclusively found in patients with Idiopathic Inflammatory Myopathies can be detected in approximately 60% of children with JDM. Anti-MDA5 antibodies, a subgroup of MSA, appear to be associated with clinically amyopathic myositis, rapidly progressive interstitial lung disease (RP-ILD) and a poor prognosis in adult East Asian dermatomyositis patients. Small studies in Japanese children with JDM have suggested similar disease phenotype. This contrasts dramatically with findings in predominantly Caucasian US adult populations where anti-MDA5 has been associated with a distinct cutaneous phenotype and no association with RP-ILD has been found.

Evidence suggests that early and aggressive treatment in Juvenile Dermatomyositis (JDM) improves outcome and prevents complications. Cyclophosphamide has been used as a second-line agent in the treatment of severe or refractory JDM. Published data on the effectiveness of cyclophosphamide in JDM are limited to a previous small case series and case reports.

Juvenile dermatomyositis (JDM) is a rare condition affecting 3 children/million/year. Muscle and skin involvement are key features. The muscle symptoms are frequently the main initial focus: formal measures (CMAS and MMT8) exist to routinely and accurately assess this component of the disease. However the involvement of skin, and its assessment, is also a vital aspect. The abbreviated Cutaneous Assessment Tool (CAT) encompassing active skin disease and skin damage, Disease Activity Score (DAS) and Myositis Intention to Treat Activity Index (MITAX), both with skin components, have all been suggested to measure skin disease in JDM; however the optimal tool is unknown.