3. Introduction
• JIA is one of chronic arthritides of
childhood
• Juvenile Idiopathic Arthritis = Juvenile
Chronic Arthritis = Juvenile Rheumatoid
Arthritis
• JIA includes groups of diseases that share
specific characteristics
20/12/2015 JIA- Mays Nairat 3
4. Etiology
• It is an autoimmune disease of unknown
etiology
• The common underlying manifestation is
the presence of chronic synovitis
20/12/2015 4JIA- Mays Nairat
5. Epidemiology
• JIA is the most common chronic rheumatologic
disease of childhood
• Prevalence 1:1000
• Two peaks; one at 1-3 yrs & one at 8-12 yrs
• Girls are affected more commonly than boys
20/12/2015 5JIA- Mays Nairat
6. Clinical Presentation
•onset of the arthritis is slow
•the actual joint swelling is often noticed
acutely
•confused with trauma
20/12/2015 6JIA- Mays Nairat
7. • Pain and stiffness in the joint that limit use,
but rarely refuses to use the joint at all
• Morning stiffness and gelling (stiffness after rest)
20/12/2015 7JIA- Mays Nairat
8. on physical examination :
• Signs of inflammation; joint tenderness,
erythema, and effusion
• Joint range of motion may be limited because of
pain, swelling, or contractures from lack of use
• Localized growth disturbances
• Leg length discrepancy if arthritis is asymmetrical
20/12/2015 8JIA- Mays Nairat
9. Eighteen-month-old girl with arthritis in her right knee.
Note the flexion contracture of that knee.
20/12/2015 9JIA- Mays Nairat
10. • All children with chronic arthritis are at risk
for chronic iridocyclitis or uveitis
• Association between uveitis and HLA-DR5/
DR6/ DR8
• Positive ANA at higher risk for chronic
uveitis
20/12/2015 10JIA- Mays Nairat
11. Highest risk group:
young girls,
With oligoarticular JIA (less than 5 joints
affected),
And Positive ANA
Incidence of uveitis 80%
20/12/2015 11JIA- Mays Nairat
14. Oligoarticular JIA
• Arthritis in fewer than five joints within 6 months
of diagnosis
• The most common form of JIA, 50%
• Two peaks; one at 1-3 yrs & one at 8-12 yrs
• Medium-sized to large joints; knee> ankle > wrist
20/12/2015 14JIA- Mays Nairat
15. Oligoarticular JIA
• Children may be otherwise well without any
evidence of systemic inflammation (fever, weight
loss, or failure to thrive) or any laboratory
evidence of systemic inflammation (elevated WBC
count or ESR)
Extended Oligoarthritis: Later development of
polyarticular disease
20/12/2015 JIA- Mays Nairat 15
17. Polyarticular JIA
• Children with arthritis in five or more joints within
the first 6 months of diagnosis
• 40% of JIA
• Symmetric arthritis, which can affect any joint but
typically involves the small joints of the hands,
feet, ankles, wrists, and knees
20/12/2015 JIA- Mays Nairat 17
18. Polyarticular JIA
• The cervical spine can be involved
• Systemic inflammation manifestation; malaise,
low-grade fever, growth retardation, anemia of
chronic disease
• Elevated markers of inflammation
• Can present at any age; however, there are 2
peaks: in early childhood (RF –ve) and
adolescence (RF +ve)
20/12/2015 JIA- Mays Nairat 18
19. 20/12/2015 JIA- Mays Nairat 19
•Patient with active polyarticular arthritis. Note swelling (effusions) of all PIP joints
in addition to boney overgrowth.
•The patient has interosseus muscle wasting
•Subluxation and ulnar deviation of the wrists are present
20. Systemic-Onset JIA
(Still’s disease)
• 10% of JIA
• They does not present with onset of arthritis
• Manifest with a typical recurring, spiking fever,
usually once or twice per day, for several weeks to
months
• accompanied by a rash, typically morbilliform and
salmon- colored
- Evanescent, occurs at time of high fever only
20/12/2015 JIA- Mays Nairat 20
24. Spondyloarthropathies
• Inflammation of the axial skeleton and sacroiliac
joints and enthesitis (inflammation of tendinous insertions)
• These include:
1. Juvenile ankylosing spondylitis
2. Psoriatic arthritis
3. Arthritis of inflammatory bowel disease
20/12/2015 JIA- Mays Nairat 24
25. Spondyloarthropathies
• Frequent presence of HLA-B27
• Need earlier Tx with TNF blockers
• They can present with peripheral arthritis,
so initially classified in other subgroups
• It is only later when the Dx becomes clear
20/12/2015 JIA- Mays Nairat 25
27. Workup
• CBC
• ESR, CRP
• ANA
• RF
• Diagnostic arthrocentesis: The synovial fluid WBC
count is typically less than 50,000 to 100,000/mm3, predominantly
lymphocytes. Gram stain and culture should be negative.
20/12/2015 JIA- Mays Nairat 27
28. • The most common radiologic finding in the
early stages of JIA is ……………………..….
20/12/2015 JIA- Mays Nairat 28
Normal bone x-ray
• Over time, periarticular osteopenia, resulting from
decreased mineralization, is most commonly found
29. 20/12/2015 JIA- Mays Nairat 29
•Severe loss of cartilage.
•Boney ankylosis involving the lateral 4 carpal bones with sparing of the pisiform.
•Erosions are present in the distal radius and ulna.
•Narrowing of the carpal/metacarpal joints is present.
30. 20/12/2015 JIA- Mays Nairat 30
Ankylosis in the cervical spine at several levels due to long-standing JIA
31. Diagnosis
• The diagnosis of JIA is established by the
presence of arthritis, the duration of the
disease for at least 6 weeks, and exclusion
of other possible diagnoses
• Children must be younger than 16 y/o at
time of onset of disease
20/12/2015 JIA- Mays Nairat 31
35. Prognosis
• The prognosis of JIA is excellent, with an
overall 85% complete remission rate
• Children with oligoarticular JIA tend to do well
• Children with polyarticular disease and
systemic-onset disease constitute most
children with functional disability
20/12/2015 JIA- Mays Nairat 35
36. • The importance of physical & occupational
therapy cannot be overstated because
when the disease remits, the physical
limitations remain with the patient into
adulthood
20/12/2015 JIA- Mays Nairat 36
