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Table 1 The main clinical and laboratorial characteristics of JIA subtypes based on International League of Associations for Rheumatology (ILAR) classification criteria. ANA – antinuclear antibodies, Anti-CCP – Anti-Cyclic Citrullinated Peptide, CRP - C-reactive protein, ERA - enthesitis-related arthritis, HLA - human leukocyte antigen system, RF – rheumatoid factor, sJIA – systemic JIA

From: Juvenile idiopathic arthritis: from aetiopathogenesis to therapeutic approaches

Subtype

Oligoarticular JIA

Polyarticular JIA RF-

Polyarticular JIA RF+

ERA

Psoriatic JIA

Systemic sJIA

Characteristic of arthritis

• ≤4 joints affected• Mainly large joints• Asymmetric, often only a single joint (knee)

• ≥5 joints affected• Symmetric or asymmetric• Small and large joints• Sometimes a cervical spine and/or temporomandibular joint

• ≥5 joints affected• Symmetric• Mainly small joints (metacarpophalangeal joints and wrists)• Erosive• Aggressive symmetric polyarthritis

• Lower limb joints affected more common• Axial involvement: sacroiliac joint, hip or shoulder

• Asymmetric arthritis• Small and large joints

• Usually arthralgias;• 30–50% chronic arthritis - slowly developed• Mostly wrists, knees, ankles joints or asymptomatic temporomandibular arthritis

Systemic manifestation

30% uveitis

10% uveitis

• Rheumatoid nodules• 10% uveitis

• Acute anterior uveitis• EnthesitisGut inflammation

• Psoriasis• Dactylitis• Onycholysis• Nail pitting• Uveitis (10–15%)

• Spiking fever• Generalized lymphadenopathy• Migratory salmon-pink rash• Serositis (pericarditis most common, then pleuritic and peritonitis)• Hepatosplenomegaly• MAS

Sex predominance

Female

Female

Female

Male

Equal

Equal

HLA genetic pre-disposition

Associated with• A2• DRB1*11• DRB1*08• DPB1*0201• DRB1*15*01• DQA1*04• DQB1*04• DRB1*13 for persistent• DRB1*01 for extended variant

Associated with• A2• DRB1*08• DPB1:03• DQA1*04• DRB1*15*01• DPB1*02*01

Associated with• DRB1*04• DRB1*01• DRB1*08• DQA1*03

Associated with• B27• DRB1*01• DQA1*01• DQB1*05

Associated with• DRB1*01• DRB1*11• DRB1*12• HLA-C*06 - biomarker for skin involvement• B27 – for sacroileitis (mostly in older age)

Associated with• DRB1:04• DQA1*01• DQB1*04• DRB1*01

Biomarkers

60% ANA+

40% ANA+

• RF+• Anti-CCP+• ANA+ in 40%

45–85% HLA-B27+

50% ANA+

Elevating level of• CRP• Ferritin• Platelets

Adult equivalent

–

Potential Seronegative Rheumatoid Arthritis

RF-positive Rheumatoid Arthritis

Spondyloarthropathies

Psoriatic Arthritis

Adult Onset Still’s Disease