Juvenile Idiopathic Arthritis Flashcards

1
Q

How is juvenile idiopathic arthritis defined?

A

A group of systemic inflammatory disorders affecting children under 16 years

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2
Q

What are the 3 main aetiological factors for juvenile idiopathic arthritis?

A

Environmental
Genetic
Immunologic

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3
Q

What’s the prevalence of juvenile idiopathic arthritis in the UK?

A

Approx. 1 in 1000

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4
Q

What are the diagnostic criteria for juvenile idiopathic arthritis?

A

Age of onset <16
Duration of disease >6 weeks
Presence of arthritis - joint swelling or 2 of; painful or limited joint motion, tenderness, warmth

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5
Q

What are the 3 major subtypes of juvenile idiopathic arthritis?

A

Pauciarticular
Polyarticular
Systemic onset

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6
Q

How long does it take after diagnosis for a subtype to be identifiable?

A

6 months

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7
Q

What is the most common type of juvenile idiopathic arthritis?

A

Pauciarticular

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8
Q

What is pauciarticular juvenile idiopathic arthritis?

A

When 4 or less joints are involved

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9
Q

What are clinical features of type 1 pauciarticular JIA?

A
Presents before 5 years
Much more common in girls
Presents with limp rather than pain
No general symptoms
Mainly affects lower limb joints
Chronic uveitis common
Asymptomatic in half of cases
Irregular iris
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10
Q

What are clinical features of type 2 pauciarticular JIA?

A
Age after 8-9
Boys much more common than girls
Occassional general symptoms
Limp 
Mainly lower limb
Hip can be affected early with rapid damage - may evolve ankylosing spondylitis or sponyloarthritis
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11
Q

What are clinical features of type 3 pauciarticular JIA?

A

Any age during childhood
Girls:boys 4:1
Asymmetric upper and lower limb arthritis
Dactylitis
Family history of psoriasis in 40% of cases

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12
Q

What is Dactylitis?

A

Inflammation of a digit

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13
Q

What is polyarticular JIA?

A

JIA affecting 5 or more joints

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14
Q

What are clinical features of rheumatic factor negative polyarticular JIA?

A
Any age but often early
Girls much more common than boys
General symptoms - low grade fever, malaise
Hepato-splenomegaly
Mild anaemia
Growth abnormalities
Symmetric large and small joint
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15
Q

What is iridocyclitis?

A

Inflammation of the iris and ciliary body

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16
Q

What are clinical features of rheumatic factor positive polyarticular JIA?

A
Late childhood 
Girls much more common than boys
General symptoms - Low grade fever, malaise, weight loss
Anaemia
Nodules
Similar to adult RA 
Erosions in x-ray occur early
17
Q

What is Still’s disease?

A

Systemic onset JIA

18
Q

What defines Still’s from other forms of JIA?

A

Extra-articular features

19
Q

What are clinical features of Still’s disease?

A

Most common 4-6years but can present throughout childhood
Girls about the same as boys
Fever of 39.5 for at least 2 weeks in late afternoon/evening returning to normal in the morning
Salmon red rash on trunk and thighs
Non tender generalised lymphadenopathy
Hepatosplenomegaly
Pericarditis
Potential pleural effusion or pulmonary fibrosis
Arthritis within 3-12 months of fever

20
Q

What is uveitis?

A

Inflammation of the uvea

21
Q

Why is uveitis important to keep in mind with JIA?

A

JIA is a common cause and it can potentially lead to blindness - can happen in any form of JIA

22
Q

How is uveitis treated?

A

Steroids - topical/intraocular/systemic
Methotrexate
Anti-TNF

23
Q

What is 1st line therapy for management of JIA?

A

Simple pain killers

NSAIDs - consider difference between half life in adults and children

24
Q

What is 2nd line therapy for management of JIA?

A
If no response to NSAIDs - 
Methotrexate
Anti-TNF
IL-1 R-antagonist
IL-6 antagonist
25
Q

What is an example of an IL-1 R-antagonist?

A

Anakinra

26
Q

What is an example of an IL-6 antagonist?

A

Tocilizumab

27
Q

When are systemic steroids used in management of JIA?

A

Systemic JIA
Serious disease complications - vasculitis, auto-immune anaemia, etc
Bridge between DMARDs
Children undergoinng surgery

28
Q

What are risks of systemic steroid use?

A

Osteoperosis
Infections
Growth abnormalities

29
Q

What are non-drug forms of management for JIA?

A

Physiotherapy

Occupational therapy

30
Q

When is intra-articular steroid use generally done?

A

Pauci-articular JIA

31
Q

What are surgical treatments for JIA?

A

Synovectomy

Reconstructive/joint replacement surgery

32
Q

What are localised growth failure problems in JIA?

A

Leg length discrepancies
Shortening of fingers, hands, forearms, toes, feet
Micrognathia

33
Q

What is micrognathia?

A

Undersized jaw

34
Q

What are generalised growth failure problems in JIA?

A

Short stature
Delayed puberty
Related to systemic steroid use and severe systemic disease