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MSK > Juvenile Idiopathic Arthritis > Flashcards

Juvenile Idiopathic Arthritis Flashcards

1
Q

What is the definition of juvenile idiopathic arthritis (JIA)?

A

Group of systemic inflammatory disorders affecting children below age of 16
Most commonly diagnosed rheumatoid disease in children
Important cause of disability and blindness

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2
Q

What is the pathogenesis of JIA?

A

Is an auto-immune condition
Multi-factorial and different of adult RA
Strong subset specific genetic markers affect immune response

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3
Q

What is the classification criteria of JIA?

A

Age of onset <16
Duration of disease >4weeks
Presence of arthritis - joint swelling or 2 of : limited or painful joint motion, tenderness or warmth

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4
Q

What are the clinical subtypes of JIA?

A

After 6 months can be identified
Oligoarticular - 4 or less joints (most common)
Polyarticular - 5 or more joints
Systemic Onset - systemically unwell

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5
Q

What will help to suspect the clinical subtypes of JIA?

A

Natural history, complications, prognosis and decide the strategy of treatment

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6
Q

What is the incidence of type 1 oligoarticular JIA?

A

Majority
Age is before 5 years, peak 1-3 years
8:1 girls to boys
Mainly lower limb (knee>ankle> hand or elbow)

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7
Q

What is the presentation of type 1 oligoarticular JIA?

A

Limp rather than a pain as mainly affects lower limbs
No constitutional manifestations
Look at eyes
+ANA (antinuclear antibody for autoimmune disease) in 40-75%

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8
Q

What is the incidence of eye disease and type 1 oligoarticular JIA?

A

Chronic uveitis in 20% of cases (95% if female and under 2 years)
Asymptomatic in 50%
Irregular iris due to posterior synechiae
Gradual progressive loss of vision

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9
Q

What is the incidence of type II oligoarticular JIA?

A

15%
Age after 8-9 years
Girl to boys 1:7

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10
Q

What is the presentation of type II oligoarticular JIA?

A

Constitutional symptoms are rare and limp due to LL affection- knee and ankle
May have erosions in heel, Achilles tendonitis and rapidly progressive inflammation in hip leading to OA

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11
Q

Describe type II oligoarticular JIA and hip joints

A

Hips can be affected early with rapid damage requiring THR early in life and enthesitis
Many have sacroiliac joints involved and may evolve to AS or spondyloarthritis
Acute iritis 10-20%

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12
Q

What is the diagnosis if present with HLA-B27 and back involvement?

A

Juvenile ankylosing spondylitis

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13
Q

What is the incidence of type III oligoarticular JIA?

A

15%
Age is any during childhood
Girls to boys 4:1

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14
Q

What is the presentation of type III oligoarticular JIA?

A

Constitutional symptoms are rare, asymmetric UL and LL arthritis and dactylitis (sausage finger or toe)

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15
Q

What is associated with type III oligoarticular JIA?

A

Arthritis can be very destructive
FH of psoriasis in 40% and possible nail pitting
These patients may develop psoriasis later in life
Chronic uveitis in 10-20%

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16
Q

What is extended oligoarthritis?

A

30% of these presenting with pauciarticular JIA can more to severe polyarticular course
Feature of poor prognosis

17
Q

What is the incidence of JIA zero negative (polyarticular)?

A

15% of JIA
Negative rheumatoid factor
Age is any age and often early
Girls to boys 9:1

18
Q

What is the presentation of JIA zero negative (polyarticular)?

A

Constitutional manifestation (low grade fever and malaise), hepato-splenomegaly, mild anaemia and growth abnormalities
Symmetric large and small joint affection - knees, wrists, ankles, MCPs, PIPs and neck
Uveitis is rare

19
Q

What is the incidence of RF positive polyarticular JIA?

A

10% of JIA
Age is later childhood (teens, 12-16 years)
Girls to boys 7:1

20
Q

What is the presentation of RF positive polyarticular arthritis?

A

Constitutional manifestations, anaemia and nodules
Can be complicated by Sjogren’s, Felty or vasculitis, AR, and pulmonary fibrosis
Similar to adult RA
Erosions in x-ray occur rarely
Uveitis rare

21
Q

Which type of JIA has the most serious morbidity and mortality?

A

Systemic onset JIA

22
Q

What is the incidence of systemic onset JIA (STILL’s disease)?

A

20% of JIA
Age is throughout childhood (4-6 years)
Girls to boys 1.5:1

23
Q

What features define systemic onset JIA?

A

Extra-articular features
Start early and disappear after 2-5 years
Fever, rash, sore throat in absence of infection, abdo pain, pleural effusion, weight loss, pericardial effusion and lymphadenopathy

24
Q

What can the fever be like in systemic onset JIA?

A

Rise to 39.5 degrees for at least 2 weeks
Late in afternoon or evening and return to normal/ subnormal in the morning
Child appears appears toxic with fever and possible chills but normal when fever goes away

25
Q

Describe the rash in systemic onset JIA

A

90%
Evanescent salmon red eruption
On trunk and thighs
Accompanies fever
Can be brought on by scratching or heat (positive Kosher’s phenomenon)

26
Q

What are the abdominal symptoms in systemic onset JIA?

A

Hepatosplenomegaly, 50-75%, abdominal pain, and possible transaminases

27
Q

What are the pulmonary symptoms of systemic onset JIA?

A

Rare, pleural effusion and pulmonary fibrosis

28
Q

Describe the arthritis in systemic onset JIA

A

75%
Within 3-12 months of onset of fever
Wrists, knees, ankles, cervical spine, hips and TMJ

29
Q

What are the investigations of JIA?

A

No diagnostic test
ESR often elevated (very high in systemic JIA but does not correlate with disease activity)
ASO often elevated with no evidence of recent infection
RF
ANA
Radiology - MRI or US

30
Q

What is the first line therapy for JIA?

A

Simple pain killers
NSAIDs
Oligoarticular can respond well to NSAIDs/ joint injections

31
Q

What is the 2nd line therapy for JIA?

A

Methotrexate
Anti-TNF therapy
IL-1 R-antagonist in refractory systemic arthritis
IL-6 antagonist for refractory systemic disease
Other biologics - Rituximab

32
Q

When are systemic steroid are used?

A

Limited indications due to serious side effects
Used in systemic JIA (control pain and fever), serious disease complications with any subtype and used as a bridge between DMARDs

33
Q

What is the risk of systemic steroids?

A

Osteoporosis, infections and growth abnormalities

34
Q

When are local steroids used?

A

Intra-articular injections
Eye disease (ANA +ve oligoarticular disease)

35
Q

What is the surgical treatment for JIA?

A

Synovectomy
Reconstructive/ joint replacement surgery

MSK (39 decks)

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