juvenile idiopathic arthritis
TRANSCRIPT
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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