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• Systemic juvenile idiopathic arthritis (sJIA) is defined by the International League of Associations for Rheumatology (ILAR) as arthritis, in children under 16 years of age, occurring in one or more joints with/or preceded by fever of at least 2 weeks’ duration that is documented to be daily (quotidian) for at least 3 days, and accompanied by one or more of the following¹:
  • Evanescent (non-fixed) erythematous rash
  • Generalised lymph node enlargement
  • Hepatomegaly and/or splenomegaly
  • Serositis 

• sJIA is one of the most severe systemic inflammatory disease in childhood and is a subtype of JIA²
  • The prevalence of JIA approximates to 100 cases per 100,000 children, of which sJIA accounts for 6–11% of cases in Europe and North America (10–20% in Caucasians)³
    

• sJIA is a chronically debilitating condition because its symptoms are recurrent, causing disability and developmental and social consequences³
  

• The disease course is heterogeneous in severity, ranging from a monocyclic course with remission within 2–4 years to a relapsing course with systemic flares and mild arthritis³
  

• Children with severe disease usually have active arthritis into adulthood despite standard therapies³

References:

1. Petty RE, et al. International league of associations for rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol 2004; 31:390–392.
2. Ravelli A, & Martini A. Juvenile idiopathic arthritis. Lancet 2007; 369:767–778.
3. Woo P, et al. Systemic juvenile idiopathic arthritis: diagnosis, management, and outcome. Nat Clin Pract Rheumatol 2006; 2:28–34.