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Muscluloskeletal Systems > Juvenile Idiopathic Arthritis > Flashcards

Juvenile Idiopathic Arthritis Flashcards

1
Q

What is the definition of Juvenile idiopathic arthritis?

A

Group of systemic inflammatory disorders affecting children below age of 16 years

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2
Q

What is the aetiology and pathogenesis of JIA?

how does this compare to RA?

A

JIA is an auto-immune disease

Aetiopathogenesis is multifactoral and different from that of adult RA

Genetic
Environmental
Immunologic

(We dont really know)

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3
Q

What is the criteria for diagnosis of JIA?

A

Age of onset 6 weeks

Joint swelling or 2 of:

  • Painful or limited joint motion
  • Tenderness
  • Warmth
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4
Q

What are the 3 major subtypes of JIA?

A

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

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5
Q

Appart from the 3 major subtypes of JIA what other clinical subtypes are there?

A

Enthesopathy related arthritis

Juvenile psoriatic arthritis:
-oligo or spondylo with psoriasis or potential psoriasis

Others:
-Unclassified under above criteria

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6
Q

How many joints are effected in Pauciarticular JIA?

A

4 or less joints

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7
Q

How many types of Pauciarticular JIA are there?

A

3 types:

  • Type 1 (25%)
  • Type II (15%)
  • Type III (15%)
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8
Q

What joints are usually affected in Pauciarticular JIA type 1?

A

Mainly lower limb

Knee > ankle > hand or elbow (hip very rare

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9
Q

What is the presentation of Pauciarticular JIA type I?

who is it most common in?

A

Limp rather than pain
-Young children dont really report pain

Preschool aged girl under 5 (peak 1-3)

M:F = 1:8

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10
Q

Why do you check ANA in pauciarticular JIA type I?

A

Positive ANA in 40-75%

Chronic uveitis in 20% of cases (95% if female

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11
Q

What is the presentation of Pauciarticular JIA type II?

who is it most common in?

A

Constitutional rare

Limp due to lower limb being more effected

Age: after 8-9

M:F = 7:1

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12
Q

What joints are most effected in pauciarticular JIA type II?

A

Mainly lower limb: knee, ankle

Hip can be affected early with rapid damage requiring total hip replacement early in life + enthesitis + many have sacroiliac joints and may evolve AS or spondyloarthritis

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13
Q

When is someone categorised as having Juvenile Ankylosing Spondylitis?

A

Those with HLA-B27 and back involvement in pauciarticular JIA type II

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14
Q

What is the presentation of pauciarticular JIA type III?

who does it commonly present in?

A
  • Constitutional rare
  • Asymmetric upper and lower limb arthritis
  • Dactylitis

Any age during childhood

M:F = 1:4

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15
Q

What family history do patients with pauciarticular JIA commonly have?

What does this mean for them?

A

FH of psoriasis in 40%
+/- nail pitting

These patients may develop psoriases later in life

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16
Q

What eye disease can occur in each type of pauciarticular JIA?

A

Type I:

  • Chronic uveitis in 20% of cases
  • If female under 2 years then 95% of cases

Type II:
-Acute iridocyclitis in 10-20%

Type III:
-Chronic iridocyclitis in 10-20%

17
Q

How many joints are affected in polyarticular JIA?

A

5 or more joints

18
Q

How does RF -ve Polyarticular JIA normally present?

who does it present in? What joints are effected?

A

Can presentt in any age, often early
M:F = 1:9

  • Constitutional manifestations (low grade fever, malaise)
  • Hepato-splenomegaly
  • Growth abnormalities

Symmetric large and small joints:
-Knees, wrists, ankles, MCPs, PIPs, neck

19
Q

What are the two types of Polyarticular JIA?

A

RF +ve

RF -ve

20
Q

How does RF +ve Polyarticular JIA normally present?

who does it present in? etc

A

Age: late childhood (teens, 12-16 years

M:F 1:7

  • Constitutional manifestations (low grade fever, malaise, weight loss)
  • Anaemia
  • Nodules
21
Q

What can RF +ve polyarticular JIA be complicated by?

What is it similar to?

A

Complicated by:

  • Sjogren’s
  • Felty or vasculitis
  • AR
  • Pulmonary fibrosis
  • AAS, CTS

Similar to adult RA but in a child

Erosions in x-ray occur early

22
Q

How common is iridocyclitis in both types of polyarteritis JIA?

A

Rare in both RF +ve and -ve

23
Q

What is systemic onset JIA also called?

A

Still’s disease

after George Frederic Still

24
Q

What is the epidaemiology of Systemic onset JIA?

A
  • Age: throughout childhood (4-6 years)
  • M:F = 1:1.5

Rare

25
Q

Extra-articular features define systemic onset JIA.

What are these features?
think of the organs effected

A
  • Pleural effusion
  • Pulmonary fibrosis
  • Polyserositis
  • Pericarditis in 36%
  • Tamponade and myocarditis

Fever
Hepatospenomegaly 50-75%
Abdominal pain
+/- transaminases

Generalised lymphadenopathy (50-75%)
-Non tender

Evanescent salmon red eruption (90%)

  • trunk and thighs
  • Positive Koebner’s phenomenon
26
Q

Explain fever in children with systemic onset JIA

How high is it?
What is its pattern?

A
  • Fever of 39.5 degrees 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 +/- chilld but looks normal when fever goes away
27
Q

What joints are effected in systemic onset JIA?

When do joint problems occur?

A

Within 3-12 months of onset of fever

Wrists, knees, ankles, cervical spine, hips and TMJ

28
Q

What tests can you carry out in juvenile idiopathic arthritis?

A

No diagnostic tests

ASO: often elevated with no evidence of recent strep infection

RF: 15-20% of children with late onset polyarticular JIA

ESR: Often elevated,

  • Very high in systemic JIA,
  • Does not correspond with disease activity

ANA: 40-60% of pauciarticular JIA

29
Q

What is the 1st line therapy in JIA?

A

Simple pain killers

NSAIDs
-Children can take these for years without any GI upset. (much more tough than adults)

30
Q

What is second line therapy in JIA?

A

Joint (steroid) injections

Methotrexate (can give much more to children than adults)

Anti TNF (if methotrexate fails)

IL-1 antagonist (Anakinra)
-Refractory systemic arthritis

IL-6 antagonist (Tocilizumab)
-Refractory systemic disease

31
Q

When are systemic steroirds used?

A

Systemic JIA (control pain and fever)

Serious disease complications with any subtype e.g. periocordial effusion, tamponade, vasculitis, severe autoimmune anaemia, severe eye disease)

As a bridge between DMARDs

Children undergoing surgery

32
Q

Why are systemic steroids only used in special indications?

A

Serious side effects:

  • Risk of osteoporosis
  • Infections
  • Growth abnormalities
33
Q

When are local steroids used in JIA?

A

Intra-articular mainly in oligo-articular JIA

Eye disease (ANA +ve oligo-articular disease)

34
Q

What surgical treatment can be considered in Juvenile Idiopatic Arthritis?

A

Synovectomy

Reconstructive/ joint replacement surgery

Muscluloskeletal Systems (29 decks)

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