Juvenile Dermatomyositis-Clinical Phenotypes

Abstract

Purpose of review: Juvenile dermatomyositis is a heterogeneous disease with variable clinical outcomes. Here, we describe the recognised subtypes of idiopathic inflammatory myositis which occur in children, with particular reference to disease-associated autoantibodies.

Recent findings: Large cohort studies have demonstrated that myositis autoantibodies are common in juvenile dermatomyositis and can be found in the majority of patients. They identify homogenous clinical subgroups and inform prognosis, particularly the risks of developing interstitial lung disease. Descriptions of immune-mediated necrotising myositis in juvenile patients have highlighted a rare but important clinical subset typically associated with severe muscle disease and treatment resistance. It is increasingly apparent that autoantibodies can provide detailed information on prognosis and the likely disease associations in those with juvenile dermatomyositis. Further work is needed to establish how this knowledge should influence our approach to treatment.

Keywords: Autoantibodies; Juvenile; Myositis; Phenotype; Subgroup.