Juvenile Idiopathic Arthritis Flashcards

1
Q

What is juvenile idiopathic arthritis?

A

Is a group of systemic inflammatory disorders affecting children < 16 years

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2
Q

What is the aetiology of JIA?

A

Aetiology:

  • Autoimmune disease with multifactorial pathogenesis
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3
Q

What does JIA stand for?

A

Juvenile idiopathic arthritis

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4
Q

What is the prevalence of JIA?

A

1/1000 children <16 years

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5
Q

What is the critieria for diagnosis of JIA?

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

What are the different clinical subtypes of JIA?

A

Pauciarticular (most prevalent)

Polyarticular

Systemic onset

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7
Q

What is the most common clinical subtype of JIA?

A

Pauciarticular

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8
Q

How many joints does pauciarticular JIA affect?

A

4 or less

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9
Q

What are the different types of pauciarticular JIA?

A
  • Type 1
    • Most prevalent type
    • Age < 5 years
    • Girls:boys 8:1
    • Presentation is limp rather than pain and stiffness, chronic uveitis, irregular iris
    • Knee > ankle > hand or elbow
  • Type 2
    • Age after 8-9
    • Girls:boys 1:7
    • Presentation – lower limp joint affected (hip can be affected), acute iridocyclitis
  • Type 3
    • Any age
    • Girls:boys 4:1
    • Presentation – constitutional rare, asymmetric upper limb or lower limb, dactylitis, chronic iridocyclitis
    • Can be associated with psoriasis (family history)
  • Extended oligoarthritis
    • Present in pauciarticular way (<4 joints) but then go onto develop polyarticular arthritis
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10
Q

What is the most prevalent type of pauciarticular JIA?

A

Type 1 pauciarticular JIA

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11
Q

Describe the epidemiology of type 1 pauciarticular JIA in terms of sex and age?

A
  • Age < 5 years
  • Girls:boys 8:1
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12
Q

Describe the epidemiology of type 2 pauciarticular JIA in terms of sex and age?

A
  • Age after 8-9
  • Girls:boys 1:7
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13
Q

Describe the epidemiology of type 3 pauciarticular JIA in terms of sex and age?

A
  • Any age
  • Girls:boys 4:1
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14
Q

What is the presentation of type 1 pauciarticular JIA?

A
  • Presentation is limp rather than pain and stiffness, chronic uveitis, irregular iris
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15
Q

What is the presentation of type 2 pauciarticular JIA?

A
  • Presentation – lower limp joint affected (hip can be affected), acute iridocyclitis
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16
Q

What is the presentation of type 3 pauciarticular JIA?

A
  • Presentation – constitutional rare, asymmetric upper limb or lower limb, dactylitis, chronic iridocyclitis
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17
Q

What type of pauiarticular JIA can be associated with psoriasis?

A

Type 3

18
Q

Where does type 1 pauciarticular JIA most commonly affect?

A
  • Knee > ankle > hand or elbow
19
Q

What is extended oligoarthritis?

A
  • Present in pauciarticular way (<4 joints) but then go onto develop polyarticular arthritis
20
Q

How many joints does polyarticular JIA affect?

A

5 or more

21
Q

What are the clinical subtypes of polyarticular JIA?

A
  • Divided into rheumatoid factor + and –
22
Q

Describe the epidemiology of polyarticular -ve JIA in terms of sex and age?

A
  • Any age
  • Girls:boys 9:1
23
Q

Describe the epidemiology of polyarticular +ve JIA in terms of sex and age?

A
  • Late childhood
  • Girls:boys 7:1
24
Q

What is the presentation of polyarticular -ve JIA?

A
  • Presentation – constitutional manifestations (fever, malaise), hepato-splenomegaly, anaemia, restriced growth, symmetric arthritis on large and small joints (knees, wrists, ankles, MCPs, PIPs, neck), uveitis rare
25
Q

What is the presentation of polyarticular +ve JIA?

A
  • Presentation – constitutional manifestations, anaemia, nodules, erosions in x-ray, uveitis rare
26
Q

What is systemic onset JIA known as?

A

STILL’s disease

27
Q

Describe the epidemiology of systemic onset JIA in terms of sex and age?

A
  • Any age
  • Girls:boys 1.5:1
28
Q

What is the characteristic presentation of systemic onset JIA?

A
  • Characteristic presentation is fever that rises daily then returns back to normal after 2 weeks with no other obvious cause, rash described as salmon red eruption on trunk and thighs, lymphadenopathy, hepatosplenomegaly with abdominal pain, serositis, lung involvement rare
29
Q

Is the arthritis in systemic onset JIA usually pauciarticular or polyarticular?

A

Polyarthritis

30
Q

What is uveitis?

A

Inflammation of the middle layer of eyeball, which consists of iris, ciliary, body and choroid

31
Q

In what clinical subtype of JIA is uveitis most common?

A
32
Q

What should every child with arthritis be screened for?

A

Eye problems (uveitis)

33
Q

What are potential complications of uveitis?

A

Is a cause of potential blindness, other complications includes:

  • Posterior synechiae
  • Cataracts
  • Band keratopathy
34
Q

What is the treatment of uveitis?

A
  • Steroids
    • First topical, sometimes systemic required
  • Mydriatic and cycloplegic agents
  • Methotrexate
  • MMF
  • Ciclosporin
  • Anti-TNF
35
Q

Describe the management of JIA?

A
  • First line therapy
    • Simple pain killers
    • NSAIDs
  • Second line therapy
    • If no response to NSAIDs/joint steroid injection
    • 1) Methotrexate
    • 2) Anti-TNF
    • 3) IL-1 R-antagonist in refractory systemic arthritis
    • 4) IL-6 antagonist for refractory systemic disease
  • Rehabilitation
    • Physiotherapy
    • Occupational therapy
  • Surgical treatment
    • Synovectomy
    • Reconstructive/joint replacement surgery
36
Q

What is the first line therapy for JIA?

A
  • First line therapy
    • Simple pain killers
    • NSAIDs
37
Q

What is the second line therapy for JIA?

A
  • Second line therapy
    • If no response to NSAIDs/joint steroid injection
    • 1) Methotrexate
    • 2) Anti-TNF
    • 3) IL-1 R-antagonist in refractory systemic arthritis
    • 4) IL-6 antagonist for refractory systemic disease
38
Q

Why do systemic steroids have limited indications in JIA?

A

Due to serious side effects

39
Q

What are indications for systemic steroids in JIA?

A
  • Systemic JIA to control pain and fever
  • Serious disease complications with any subtype
  • As a bridge between DMARDs
40
Q

What kinds of growth failure can occur in JIA?

A
  • Localised growth failure
    • Leg length discrepancies
    • Shortening of fingers, hands, forearms, toes, feet
    • Micrognathia (poor growth of mandible)
      • Implications for tooth development and appearance
  • Generalised growth failure
    • Related to severe systemic disease
    • Short stature
    • Delayed puberty
41
Q

What is micrognathia?

A
  • Micrognathia (poor growth of mandible)
    • Implications for tooth development and appearance