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Juvenile Idiopathic Arthritis

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Definition

• “Definite arthritis of unknown origin that begins before the age of 16 years and persists for at least 6 weeks”

Terminology• JRA/JCA/JIA

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Juvenile Idiopathic Arthritis

• Most common chronic rheumatic disease of childhood.

• Group of related, genetically heterogeneous, phenotypically diverse immunoinflammatory disorders affecting joints and other structures.

• True frequency is not known 1 to 20 per 100,000 population.

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Clinical Manifestations

• Swelling of a joint/synovial hypertrophy• Limited range of motion• Tenderness/Warmth/redness of a joint• Gait disturbance

• Fever, rash, serositis, red eyes• Anorexia, weight loss , and growth failure• Sleep disturbances, Fatigue

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Systemic JIA

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Polyarticular JIA

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Radiograph of Hands

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Oligoarticular JIA

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Oligoarticular JIA - Radiograph of knees

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Juvenile psoriatic arthritis

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JIA Enthesitis related arthritis

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JIA SubgroupsJIA subtype % Age Girls% Typical Pattern HLA B27

Systemic arthritis

4-7 4-7 48-70 Any or none 4-11

Oligoarthritis 46-54 4-5 66-78 Large joints, asymmetric 11-17

RF-negative polyarthritis

13-21 4-7 76-80 Any, often symmetric 10-11

RF-positive polyarthritis

1-4 11-12 83-92 typically symmetric arthritis 8-18

Psoriatic arthritis

3-7 8-10 57-69 Spine, lower extremities, distal interphalangealjoints

8-21

Enthesitis-related arthritis

4-14 10-12 9-38 Spine, sacroiliac, lower extremities, thoracic cagejoints

67-76

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Prognosis

• Common misconception - “childhood arthritis would disappear in adulthood”

• Clinical remission in 40-60% (over 10 – 28 yrs) Systemic-onset JIA 0-50% Oligoarthritiscular JIA 50- 80% Polyarticular JIA 20-30% ERA 0- 30% PSA 30- 40%

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ILAR(2001) criteria - juvenile idiopathic arthritis

1. Systemic2. Oligoarthritis

a. Persistentb. Extended

3. Polyarthritis (rheumatoid factor negative)4. Polyarthritis (rheumatoid factor positive)5. Psoriatic arthritis6. Enthesitis-related arthritis7. Undifferentiated arthritis

a. Fits no other categoryb. Fits more than one category

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Complications

Growth retardation and osteopenia

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TMJ involvement with growth failure of lower jaw

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Sequelae of chronic uveitis

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Other Complications

• Localised growth disturbances• Sexual maturation, sleep disturbance and

fatigue• Macrophage activation syndrome• Amylodosis• Drugs related side effects

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Diagnosis

• History and clinical examination• Laboratory evaluation– Acute phase reactants– ANA/Rheumatoid factor– Fluid analysis

• Imaging– Radiographs– MRI/ CT/USG

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Treatment - Drugs

• Steroids – intra-articular /oral• Non steroidal anti inflammatory drugs• DMARDS – Methotrexate, sulfasalazine,

leflunomide, cyclosporine,thalidomide• Biologicals – TNF alpha blockers, IL-1, IL-6

blockers, costimulation inhibitors ,Rituximab, IVIg.

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Supportive care

• Physical therapy• Occupational therapy

• Psychological development and growth– Peer group relationship and schooling– Nutrition– Parent counselling

• Coordinated care– Pediatric rheumatologist– Opthalmology consultations

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Surgical management

• Need for surgical therapy may be decreasing as a result of improved medical management

• Epiphysiodesis• ? Synovectomy• Soft tissue surgery• Arthroplasty

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Take home messages

• JIA is more than just one disease• Arthritis is a clinical diagnosis• Early aggressive therapy according to subtype

of JIA (uveitis).• Bone health, physical and psychological

growth.

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Thank you