Juevenile Idiopathic Arthritis Flashcards

1
Q

Juvenile idiopathic arthritis

A
  • JIA is an auto-immune disease.
  • Etiopathogenesis is multi-factorial and different from that of adult RA
  • prevalence 1/1000 under 16 years
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2
Q

JIA characterised by:

A
  • Age of onset <16 years
  • Duration of disease >6 weeks
  • Joint swelling or 2 of the following:
    - Painful or limited joint motion
    - Tenderness
    - Warmth
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3
Q

After 6 months, 3 major subtypes can be identified:

A
  • pauciarticular
  • polyarthritis
  • systemic onset
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4
Q

Pauciarticular JIA

A
  • 4 or less joints
  • 55% - most common
  • Type I, Type II, Type III
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5
Q

Pauciarticular JIA Type I

A
  • 25%
  • age <5
  • girls>boys
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6
Q

Pauciarticular JIA Type I presentation

A
  • limp rather than pain
  • no constitutional manifestations
  • mainly LL joints
  • knee > ankle > hand or elbow
  • +ve ANA in 40-50%
  • chronic uveitis in 20% of cases
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7
Q

Pauciarticular JIA Type II

A
  • 15%
  • age >8
  • boys>girls
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8
Q

Pauciarticular JIA Type II presentation

A
  • constitutional rare
  • limp due to LL affection
  • mainly LL joints: knee, ankle
  • hip can be affected early with rapid damage requiring THR early in life
  • those with HLA-B27 and back involvement will be categorised as Juvenile Ankylosing spondylitis
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9
Q

Pauciarticular JIA Type III

A
  • 15%
  • age: any age during childhood
  • girls>boys
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10
Q

Pauciarticular JIA Type III presentation

A
  • constitutional rare
  • asymmetric upper limb and lower limb arthritis
  • dactylitis
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11
Q

Pauciarticular JIA Type III prognosis

A
  • arthritis can be very destructive
  • +/- nail pitting
  • family history of psoriasis in 40%
  • these patients may develop psoriasis later in life
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12
Q

Polyarticular JIA

A
  • 5 or more joints
  • 25% second most common
  • RF -ve
  • RF +ve
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13
Q

Polyarthritic JIA RF -ve

A
  • 15% of JIA
  • age: any age
  • girls>boys
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14
Q

Polyarthritic JIA RF -ve presentation

A
  • constitutional manifestations (low grade fever, malaise, weight loss)
  • hepatosplenomegaly
  • mild anaemia
  • growth abnormalities
  • symmetric largevand small joints affection
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15
Q

Polyarthritic JIA RF +ve

A
  • 10% of JIA
  • age: 12-16 years
  • girls>boys
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16
Q

Polyarthritic JIA RF +ve presentation

A
  • constitutional manifestations (low grade fever, malaise, weight loss)
  • anaemia
  • nodules
  • similar to adult RA but in a child
  • erosions in X-ray occur early
17
Q

Systemic onset JIA

A
  • least common
  • most serious short and long term morbidity and mortality
  • age: 4-6 years
  • girls>boys
  • extra-articulate features define the disease
    - fever
    - rash
    - lymph nodes
    - abdominal
    - serositis
    - pulmonary
    - arthritis
18
Q

Systemic onset JIA fever

A
  • rises to 39.5C daily for at least 2 weeks
  • late in afternoon or evening and returns to normal or subnormal in the morning
  • child appears toxic with fever and chills but looks normal when fever goes away
19
Q

Systemic onset JIA rash

A
  • 90%
  • evanescet salmon red eruptions
  • on truck and thighs
  • accompanies fever
  • can be brought by scratching (+ve Kowbner’s phenomenon)
20
Q

Systemic onset JIA lymph nodes

A
  • 50-75%
  • generalised lymphadenopathy
  • non tender
21
Q

Systemic onset JIA abdominal

A
  • hepatosplenomegaly
  • 50-75%
  • abdominal pain
  • +/- transaminases
22
Q

Systemic onset JIA serositis

A
  • polyserositis
  • pericarditis in 36%
  • tamponade and myocarditis rare
23
Q

Systemic onset JIA pulmonary

A
  • rare
  • pleural effusion
  • pulmonary fibrosis
24
Q

Systemic onset JIA arthritis

A
  • 75%
  • within 3-12 months of onset of fever
  • wrists, knees, ankles, cervical spine, hips and TMJ
25
Q

Uveitis

A
  • JIA most common cause of non-infectious cause of uveitis
  • can affect all JIA groups
  • 15-24% patients with JIA develop uveitis early in disease course
  • screening- important for all JIA
26
Q

Uveitis first line treatment

A
  • simple pain killers
  • NSAIDs
  • intra-articular injections
27
Q

Uveitis second line treatment

A

-methotrexate
-anti-TNF Rex (all 3) in methotrexate failure
In refractory systemic arthritis:
-IL-1 R-antagonist
-IL-6 antagonist

28
Q

When should systemic steroids be used?

A
  • limited indications due to serious side effects
  • systemic JIA
  • serious disease complications with any subtype
  • as a bridge between DMARDs
  • children undergoing surgery
  • risk of osteoporosis
29
Q

JIA rehabilitation

A
  • physiotherapy

- occupational therapy

30
Q

Localised growth failure

A
  • leg length discrepancies
  • shortening of fingers, hands, forearms, toes, feet
  • micrognathia
31
Q

Generalised growth failure

A
  • relate to severe systemic disease
  • short stature
  • delayed puberty
  • systemic steroids