Developments in the Classification and Treatment of the Juvenile Idiopathic Inflammatory Myopathies

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Key points

  • •

    Juvenile dermatomyositis is the most common clinicopathologic form of the juvenile idiopathic inflammatory myopathies (IIM), but juvenile polymyositis and overlap myositis are additional distinct groups with higher morbidity and mortality rates.

  • •

    Myositis-specific and myositis-associated autoantibodies define several distinct serologic subgroups of juvenile myositis, which share many features with those of patients with adult IIM with the same autoantibodies. Of these, anti-p155/140 and anti-MJ

Clinicopathologic classification of JIIM

The recent classification has been achieved by large registry studies that have better defined the features of the most common phenotypes and additional case series and reports that have enhanced our understanding of the spectrum of the rarer clinical phenotypes. The classification of JIIM, based on clinical and histologic features, has been recognized both in adults and children with myositis, with the finding that the same clinical subgroups exist in JIIM as adult IIM but with different

Serologic classification of JIIM

An alternative classification of the JIM is by the presence of myositis autoantibodies. Two classes of autoantibodies include MSAs, which are present almost exclusively in patients with myositis, and MAAs, which are present in patients with myositis and in patients with other autoimmune diseases (Table 2). As the recognition of myositis autoantibodies has increased recently, this has enabled the identification of a myositis autoantibody in approximately 70% of patients with JIIM.5 These

Treatment approaches for JDM

The development of new therapies for JIIM has been historically hampered not only by the rarity of these conditions but also by a lack of common outcome measures and controlled clinical trials and by vastly different approaches to therapy by geographic region and specialty. Important advances in the past decade should help provide a foundation for the development of new therapies for JIIM. Two large international clinical research consortia, the International Myositis Assessment and Clinical

Summary

The JIIM are heterogeneous diseases defined by clinicopathologic groups and myositis autoantibody phenotypes, which segregate patients sharing similar demographic and clinical features, laboratory abnormalities, and prognoses into more homogeneous subsets. These phenotypes are similar in children and adults within the same subgroup. Treatments have advanced to include more aggressive approaches in the initial treatment of JDM, typically consisting of daily oral prednisone and methotrexate,

Acknowledgments

The authors thank Drs Adriana Almeida de Jesus and Hanna Kim for the critical reading of the article, Dr Frederick Miller for his support of this research on natural history and therapy for juvenile myositis, and the CureJM Foundation for their support of the George Washington University Myositis Clinic. The authors also thank the patients and physicians who have participated in the Childhood Myositis Heterogeneity Study and the George Washington University Myositis Clinic for helping to better

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    This research was supported in part by the Intramural Research Program of the NIH, National Institute of Environmental Health. The authors have no conflicts of interest.

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