Early recognition of juvenile dermatomyositis

Abstract

Juvenile dermatomyositis (JDM) is one of the most disabling chronic inflammatory conditions in children. As with many such disorders, children may have periods of non-specific symptoms before a definitive diagnosis is established. Pachman et al use data accumulated from a national registry of JDM to address the impact of the duration of such untreated symptoms before diagnosis on the clinical and laboratory features of the disease at the time of definitive diagnosis. The study drew from a cohort of 166 children, a powerful number given the rarity of JDM. One of the most striking findings of this study is the tendency of muscle enzyme determinations to fall within the normal range with increasing duration of untreated symptoms. Because elevation in the concentration of enzymes such as CK, aldolase, LDH, and SGOT/AST are included among the diagnostic criteria for JDM, it is important to consider duration of symptoms in interpreting these findings. In addition to providing useful information to the specialist considering JDM in a child, this important study reminds the generalist of the usual presentation and findings in this rare but important disorder.