Juvenile dermatomyositis: extramuscular manifestations and their management.

Purpose Of Review: Over the last few years, there have been several collaborative efforts focusing on how to assess the many aspects of juvenile dermatomyositis (JDM). It has become apparent that skin manifestations are an important part of the disease process that need to be evaluated, treated and controlled. Other extramuscular manifestations, such as calcinosis, are particularly difficult to treat, and studies are aimed at trying to elucidate predictors.

Recent Findings: Larger cohort studies have enabled work on predictors of disease course and severity to be carried out. These include new autoantibodies in JDM (p140, which appears to have an association with calcinosis and p155 with lipodystrophy), cytokine polymorphisms, which appear to be risk factors for developing JDM or for developing complications such as calcinosis, and the clinical findings of persistent nailfold capillary changes that are associated with a chronic disease course. Tools for more detailed assessment of skin disease have been developed and evaluated.

Summary: Improved collection of standardized clinical data regarding extramuscular manifestations of JDM should allow researchers to continue elucidating the prognostic factors in this rare disease and assist multicentred trials in the evaluation of different treatment options.