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Pathophysiology of sJIA

  • The aetiology of sJIA is unknown, although many clinical features are similar to those seen in autoinflammatory syndromes, including1:
    • Fever
    • Multisystem involvement
    • Polycyclic course in some patients
  • Absence of adaptive immunity involvment1,2
    • Autoreactive lymphocytes are usually undetected
    • Lack of human leukocyte antigen (HLA) association
    • Rheumatoid factor (RF) is usually absent
    • Patients rarely have positive anti-nuclear antibody (ANA) test
  • Innate immunity and complement genes are differentially expressed2
  • As with other autoinflammatory syndromes, sJIA patients are at risk of amyloidosis1

References

  1. Vastert S, et al. systemic JIA: New developments in the understanding of the pathophysiology and therapy. Best Pract Res Clin Rheumatol 2009; 23:655–644.
  2. Woo P & Colbert R. An overview of genetics of paediatric rheumatic diseases. Best Pract Res Clin Rheumatol 2009; 23:589–597.
    3.