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Table 3 Differences in the pathogenesis between oligoarticular, polyarticular rheumatoid factor (RF)-negative and positive, systemic (sJIA), psoriatic arthritis and enthesitis-related arthritis (ERA). As an autoinflammatory disease sJIA is different in pathogenesis, clinical manifestations, and therapeutic strategy compared to non-systemic subtypes of JIA. ANA - Antinuclear antibodies, anti-MCV - antibodies against mutated citrullinated vimentin, anti-CCP - anti-cyclic citrullinated peptide, IL - interleukin, MIF - macrophage migration inhibitory factor, PsJIA – psoriatic JIA, RF – rheumatoid factor, sJIA – systemic JIA, TNF - tumour necrosis factor

From: Juvenile idiopathic arthritis: from aetiopathogenesis to therapeutic approaches

  Oligoarticular JIA Polyarticular JIA ERA Psoriatic JIA sJIA
Type of disease Autoimmune Autoimmune Autoimmune Early-onset PsJIA – autoimmune, while
late-onset PsJIA – autoinflammatory
Autoinflammatory
Immune system mainly involved in pathogenesis Adaptive immune system Adaptive immune system Adaptive immune system Adaptive immune system in early-onset PsJIA
Innate immune response in late-onset PsJIA
Innate immune system
Gene association MHC class II MHC class II HLA-B27 HLA-B/C, HLAB, IL12B, IL23R, TNP1, TRAF3IP3, REL
HLA-B27 in late-onset PsJIA
TNF, IL6, IL10, MIF, IL1
Antibodies ANA ANA
RF, anti-CCP, anti-MCV – for RF+ JIA
ANA may be positive in some cases ANA in the early-onset PsJIA
Predominant effector cells CD4+, CD8+ T-cells, neutrophils CD4+, CD8+ T-cells γδT-cells, Th17 cells Th1 and Th17 cells subsets, macrophages Monocytes, macrophages, neutrophils
Key moment in pathogenesis Imbalance between inflammatoryTh1/Th17 and Treg cells Imbalance between pro-inflammatory Th1/Th17 and Treg cells HLA-B27 involved in presentation of unidentified arthritogenic peptide caused T-cells activation and induction of endoplasmic reticulum stress Autoinflammatory activation at the synovial-entheseal complex
Autoimmune processes in extra-articular tissues
Abnormal activation of phagocytes leads to hypersecretion of pro-inflammatory cytokines
Main pro-inflammatory cytokines TNFα, IL17, IFNγ TNFα, IL17, IL33, IFNγ TNFα, IL17, IL23 IL17, IL23 IL1, IL6, IL18, IL37, LRG and ADA2
Main treatment targets Inhibition of T-cell proliferation, rarely anti-TNFα therapy is needed Inhibition of T-cell proliferation, block of TNFα Block of TNFα Inhibition of T-cell proliferation, block of TNFα Block of IL1 and IL6 signalling pathway