Systemic Juvenile Idiopathic Arthritis

Pediatr Clin North Am. 2018 Aug;65(4):691-709. doi: 10.1016/j.pcl.2018.04.005.

Abstract

Systemic juvenile idiopathic arthritis (sJIA) is a distinctive subtype of juvenile idiopathic arthritis, characterized by fever and arthritis, often accompanied by rash, sometimes by generalized lymphadenopathy, hepatosplenomegaly, and serositis. The diagnosis requires adequate exclusion of infectious, oncologic, autoimmune, and autoinflammatory diseases. Macrophage activation syndrome, a serious and potentially fatal complication of sJIA, requires prompt evaluation and treatment. Newer biologic agents, particularly interleukin-1 and interleukin-6 inhibitors, are highly effective and have transformed the treatment approach by reducing the use of systemic glucocorticoids. Primary care providers have a crucial role in monitoring children with sJIA for disease-related complications and medication-related adverse events.

Keywords: Juvenile idiopathic arthritis; Macrophage activation syndrome; Pediatrics; Rheumatology; Systemic juvenile idiopathic arthritis.

Publication types

  • Review

MeSH terms

  • Arthritis, Juvenile* / complications
  • Arthritis, Juvenile* / diagnosis
  • Arthritis, Juvenile* / drug therapy
  • Biological Products / adverse effects
  • Biological Products / therapeutic use*
  • Child
  • Glucocorticoids / adverse effects
  • Glucocorticoids / therapeutic use
  • Humans
  • Macrophage Activation Syndrome / etiology

Substances

  • Biological Products
  • Glucocorticoids