Juvenile idiopathic arthritis
-
Upload
upender-satelli -
Category
Health & Medicine
-
view
316 -
download
1
Transcript of Juvenile idiopathic arthritis
1
Juvenile Idiopathic Arthritis
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
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.
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
5
Systemic JIA
6
Polyarticular JIA
7
Radiograph of Hands
8
Oligoarticular JIA
9
Oligoarticular JIA - Radiograph of knees
10
Juvenile psoriatic arthritis
11
12
JIA Enthesitis related arthritis
13
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
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%
15
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
16
Complications
Growth retardation and osteopenia
17
TMJ involvement with growth failure of lower jaw
18
Sequelae of chronic uveitis
19
Other Complications
• Localised growth disturbances• Sexual maturation, sleep disturbance and
fatigue• Macrophage activation syndrome• Amylodosis• Drugs related side effects
20
Diagnosis
• History and clinical examination• Laboratory evaluation– Acute phase reactants– ANA/Rheumatoid factor– Fluid analysis
• Imaging– Radiographs– MRI/ CT/USG
21
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.
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
23
Surgical management
• Need for surgical therapy may be decreasing as a result of improved medical management
• Epiphysiodesis• ? Synovectomy• Soft tissue surgery• Arthroplasty
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.
25
Thank you