The pathogenesis of oligoarticular/polyarticular vs systemic juvenile idiopathic arthritis

Yu Tsan Lin, Chen Ti Wang, M. Eric Gershwin, Bor Luen Chiang

Research output: Contribution to journalArticlepeer-review

118 Scopus citations


Juvenile idiopathic arthritis (JIA) has had a long and difficult problem with classification. It is clearly a heterogeneous and multi-factorial autoimmune disease but all too often the distinctions among subtypes were unclear. In fact, there is now increasing evidence of a distinct pathogenesis of oligo/polyarticular JIA compared to systemic JIA. Oligo/polyarticular JIA is an antigen-driven lymphocyte-mediated autoimmune disease with abnormality in the adaptive immune system. Cartilage-derived auto-antigens activate autoreactive T cells including Th1 and Th17 cells with production of pro-inflammatory cytokines IFN-γ and IL-17. On the other hand, the inhibition of regulatory T (Treg) cells including natural Foxp3+ Treg and self-heat shock protein-induced Treg cells with decreased anti-inflammatory cytokine IL-10 results in the loss of immune tolerance. Imbalance between autoreactive Th1/Th17 and Treg cells leads to the failure of T cell tolerance to self-antigens, which contributes to the synovial inflammation of oligo/polyarticular JIA. By contrast, systemic JIA is an autoinflammatory disease with abnormality in the innate immune system. A loss of control of the alternative secretory pathway leading to aberrant activation of phagocytes including monocytes, macrophages and neutrophils seems to be involved in the release of pro-inflammatory cytokines IL-1, IL-6, IL-18 and pro-inflammatory S100-proteins, which contribute to the multisystem inflammation of systemic JIA. Markedly distinct pathogenesis of oligo/polyarticular JIA and systemic JIA implies that they might need different treatment strategies.

Original languageEnglish (US)
Pages (from-to)482-489
Number of pages8
JournalAutoimmunity Reviews
Issue number8
StatePublished - Jun 2011


  • Autoinflammatory disease
  • Oligo/polyarticular juvenile idiopathic arthritis
  • Phagocytes
  • Regulatory T cells
  • Systemic juvenile idiopathic arthritis
  • Th17 cells

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy


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