Juvenile Idiopathic Inflammatory Myopathies

Pediatr Clin North Am. 2018 Aug;65(4):739-756. doi: 10.1016/j.pcl.2018.04.006.

Abstract

The juvenile idiopathic inflammatory myopathies (JIIM) are a group of rare, chronic, autoimmune illnesses that affect muscle and, to a lesser extent, skin. The presence of new-onset weakness and, in juvenile dermatomyositis, typical rahes, should lead to consideration of these diagnoses. Careful evaluation to exclude alternative diagnoses is needed. Investigations include a variety of blood tests, imaging, and possibly muscle biopsy. Validated clinical assessments are available for monitoring. Standard treatment includes corticosteroids and methotrexate and often extends beyond 1 year. Outcomes are generally good, but disease persistence remains problematic. Early involvement of providers with expertise in JIIM is essential.

Keywords: Juvenile dermatomyositis; Juvenile idiopathic inflammatory myopathy; Juvenile polymyositis.

Publication types

  • Review

MeSH terms

  • Age of Onset
  • Child
  • Dermatologic Agents / therapeutic use
  • Dermatomyositis / diagnosis
  • Dermatomyositis / drug therapy
  • Diagnosis, Differential
  • Drug Therapy, Combination
  • Female
  • Glucocorticoids / therapeutic use
  • Humans
  • Incidence
  • Male
  • Methotrexate / therapeutic use
  • Myositis / diagnosis*
  • Myositis / drug therapy*
  • Myositis / epidemiology
  • Practice Guidelines as Topic

Substances

  • Dermatologic Agents
  • Glucocorticoids
  • Methotrexate