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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