Juvenile idiopathic arthritis

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  • 1. Juvenile Idiopathic Arthritis 1
  • 2. 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 2
  • 3. 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. 3
  • 4. 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 4
  • 5. Systemic JIA 5
  • 6. Polyarticular JIA 6
  • 7. Radiograph of Hands 7
  • 8. Oligoarticular JIA 8
  • 9. Oligoarticular JIA - Radiograph of knees 9
  • 10. Juvenile psoriatic arthritis 10
  • 11. 11
  • 12. JIA Enthesitis related arthritis 12
  • 13. JIA Subgroups JIA 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 interphalangeal joints 8-21 Enthesitis- related arthritis 4-14 10-12 9-38 Spine, sacroiliac, lower extremities, thoracic cage joints 67-76 13
  • 14. 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% 14
  • 15. ILAR(2001) criteria - juvenile idiopathic arthritis 1. Systemic 2. Oligoarthritis a. Persistent b. Extended 3. Polyarthritis (rheumatoid factor negative) 4. Polyarthritis (rheumatoid factor positive) 5. Psoriatic arthritis 6. Enthesitis-related arthritis 7. Undifferentiated arthritis a. Fits no other category b. Fits more than one category 15
  • 16. Complications Growth retardation and osteopenia 16
  • 17. TMJ involvement with growth failure of lower jaw 17
  • 18. Sequelae of chronic uveitis 18
  • 19. Other Complications • Localised growth disturbances • Sexual maturation, sleep disturbance and fatigue • Macrophage activation syndrome • Amylodosis • Drugs related side effects 19
  • 20. Diagnosis • History and clinical examination • Laboratory evaluation – Acute phase reactants – ANA/Rheumatoid factor – Fluid analysis • Imaging – Radiographs – MRI/ CT/USG 20
  • 21. Treatment - Drugs • Steroids – intra-articular /oral • Non steroidal anti inflammatory drugs • DMARDS – Methotrexate, sulfasalazine, leflunomide, cycl osporine,thalidomide • Biologicals – TNF alpha blockers, IL-1, IL-6 blockers, costimulation inhibitors ,Rituximab, IVIg. 21
  • 22. Supportive care • Physical therapy • Occupational therapy • Psychological development and growth – Peer group relationship and schooling – Nutrition – Parent counselling • Coordinated care – Pediatric rheumatologist – Opthalmology consultations 22
  • 23. Surgical management • Need for surgical therapy may be decreasing as a result of improved medical management • Epiphysiodesis • ? Synovectomy • Soft tissue surgery • Arthroplasty 23
  • 24. 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. 24
  • 25. Thank you 25