Juvenile Idiopathic Arthritis Flashcards

1
Q

What is juvenile idiopathic arthritis?

A

A group of systemic inflammatory disorders affecting children below the age of 16

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2
Q

What kind of disease is JIA?

A

Autoimmune

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3
Q

Causes of JIA

A

Genetic
Environmental
Immunologic

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4
Q

Criteria for diagnosis of JIA

A
  1. age of onset < 16 y/o
  2. Duration of disease > 6 weeks
  3. Presence of arthritis i.e. joint swelling or 2 of the following
    - painful or limited joint movement
    - tenderness
    - warmth
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5
Q

After 6 months, what 3 types of JIA can be identified?

A

Pauciarticular (55%)
Polyarticular (25%)
Systemic onset (20%)

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6
Q

Which type of JIA is most dangerous?

A

Systemic onset

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7
Q

Clinical subtypes of JIA

A

Enthesopathy related arthritis
Juvenile psoriatic arthritis
Others

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8
Q

Features of paucarticular JIA

A

4 or less joints

3 types

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9
Q

Features of type I paucarticular JIA

A
Mainly LL joints
Knee > ankle > hand or elbow (hip very rare) 
1-3 years peak - up to 5 y/o 
G > B 8:1 - presents in girls 
\+ ANA in 40-75%
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10
Q

Presentation of type I pauarticular JIA

A

Limp rather than pain
abnormal gate
systemically fine

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11
Q

What is ANA?

A

A marker of autoimmune connective tissue disease in adults

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12
Q

+ve ANA in type I pauarticular JIA means they are at risk of what?

A

Chronic uveitis in 20% (95% if < 2 y/o)
Asymptomatic in 50%
Irregular iris due to posterior synachaeia

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13
Q

Features of type II pauarticular JIA?

A

> 8-9 y/o

G < B 1:7 - presents In boys

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14
Q

Those with HLA-B27 and back involvement will be categorized as what?

A

Juvenile ankylosing spondylitis

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15
Q

Presentation of type II pauarticular JIA

A
Acute iridocyclitis in 10-20%
Mainly LL joints
Constitutional symptoms rare
Asymmetrical UL + LL arthritis 
Dactylitis 
Hips can have rapid damage early on requiring THR early in life and entesitis and many have sacroiliac joints and may involve AS or spondyloarthritis
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16
Q

Features of Type III paucarticular JIA

A

Boys and girls
FH of psoriasis in 40% +/- nail pitting
- these patients may develop psoriasis later on in life
Chronic iridocyclitis in 10-20%
Extended oligoarthritis (30% develop this)

17
Q

Features of polyarticular JIA

A

5 or more joints
Second most common type
Two types

18
Q

2 types of polyarticular JIA

A

RF -ve

RF +ve

19
Q

features of RF -ve polyarticular JIA

A

JIA
Any age, often early
G > B 9:1

20
Q

Presentation of RF -ve polyarticular JIA

A
Idiocyclitis rare
Growth abnormalities
Constitutional manifestations
- low grade fever 
- malaise 
Hepato-splenomegaly 
Mild anaemia 
Symmetric large and small joints affected
- knees
- wrists 
- ankles
- MCP
Iridycitis rare
21
Q

Features of RF +ve polyarticular JIA

A

Late childhood/teens (12-16 y/o)
F > M 7:1
Similar to adult RA but in a child

22
Q

Presentation of JIA

A
Erosions in X ray occur early 
Constitutional symptoms 
- low grade fever
- malaise
- weight loss
Anaemia
Nodules
iridiocyclitis rare
23
Q

RF +ve JIA polyarticular can be complicated by….

A
Sjogrens
Felty 
vasculitis 
AR
pulmonary fibrosis
AAS
CTS
24
Q

Features of systemic onset JIA

A

Less common
Extra-articular features define the disease
- start early and disappear after 2-5 years
4-6 y/o
G > B 1.5:1

25
Q

Presentation of systemic onset JIA

A
fever 
- rise to 39.5C daily for at least 2 weeks
- persistent
- late in afternoon and evening and returns to (sub)normal in the morning 
Rash (90%)
- evanescent salmon red 
- on trunk and thighs 
- accompanies fever and goes when fever goes
- can be brought on by scratching (+ve kobeners phenomenon) 
Lymph nodes (50-75%)
- non tender
- generalised lymphadenopathy 
Abdominal 
- pain 
- hepatosplenomegaly 
- +/- transaminases 
arthritis (75%)
- within 3-12 months of onset of fever
- Wrists, knees, ankles, cervical spine
26
Q

Diagnosis of JIA

A

NO DIAGNOSTIC TESTS
ESR often elevated
- very high in systemic JIA, does not correlate with disease activity
ASO
- often elevated with no evidence of strep infection
RF - 15-20% of children have late onset
ANA: 40-60% of pauarticular JIA

27
Q

Treatment of JIA

A

1st line
- simple painkillers
- NSAIDs
- steroids I/A
2nd line (if first lines dont work)
- methotrexate
- anti-TNF (If methotrexate doesn’t work)
- IRL R-antagonist (for refractory systemic arthritis)
- IL-6 antagonist (for refractory systemic disease)
Systemic steroids
- limited indications due to S/Es
Local steroids (Intrarticular/oligoarticular JIA)
Topical steroids (rehab)
Surgery (synovectomy, joints replacements)

28
Q

When are steroids used?

A

Systemic JIA (control pain and fever)
Serious disease complications e.g. pericardial effusion, tamponade, vasculitis
As a bridge between DMARDs
Children undergoing surgery

29
Q

Risks of steroid use in children

A

Osteoporosis
Infections
Growth abnormalities