Abstract
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 language | English (US) |
|---|---|
| Pages (from-to) | 482-489 |
| Number of pages | 8 |
| Journal | Autoimmunity Reviews |
| Volume | 10 |
| Issue number | 8 |
| DOIs | |
| State | Published - Jun 2011 |
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Keywords
- 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
Cite this
The pathogenesis of oligoarticular/polyarticular vs systemic juvenile idiopathic arthritis. / Lin, Yu Tsan; Wang, Chen Ti; Gershwin, M. Eric; Chiang, Bor Luen.
In: Autoimmunity Reviews, Vol. 10, No. 8, 06.2011, p. 482-489.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - The pathogenesis of oligoarticular/polyarticular vs systemic juvenile idiopathic arthritis
AU - Lin, Yu Tsan
AU - Wang, Chen Ti
AU - Gershwin, M. Eric
AU - Chiang, Bor Luen
PY - 2011/6
Y1 - 2011/6
N2 - 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.
AB - 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.
KW - Autoinflammatory disease
KW - Oligo/polyarticular juvenile idiopathic arthritis
KW - Phagocytes
KW - Regulatory T cells
KW - Systemic juvenile idiopathic arthritis
KW - Th17 cells
UR - http://www.scopus.com/inward/record.url?scp=79957643739&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=79957643739&partnerID=8YFLogxK
U2 - 10.1016/j.autrev.2011.02.001
DO - 10.1016/j.autrev.2011.02.001
M3 - Article
C2 - 21320644
AN - SCOPUS:79957643739
VL - 10
SP - 482
EP - 489
JO - Autoimmunity Reviews
JF - Autoimmunity Reviews
SN - 1568-9972
IS - 8
ER -