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Systems: MSK AB > Juvenile Idiopathic Arthritis > Flashcards

Juvenile Idiopathic Arthritis Flashcards

1
Q

What is juvenile idiopathic arthritis?

A
  • Group of systemic inflammatory disorders affecting children below age of 16 years.
  • The most commonly diagnosed Rheumatic disease in children.
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2
Q

What is juvenile idiopathic arthritis an important cause of?

A

Disability and blindness

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3
Q

What is the aetiology and pathogenesis of JIA?

A
  • JIA is an auto-immune disease.
  • Etiopathogenesis is multi-factorial and different from that of adult RA.
  • Strong subset-specific genetic markers may affect immune response
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4
Q

What is the criteria 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 movement
  • Tenderness
  • Warmth
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5
Q

When can subtypes of JIA be identified?

A

After 6 months

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6
Q

What are the 3 major subtypes of JIA?

A
  • Pauciarticular 55%
  • Polyarticular 25%
  • Systemic onset 20%
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7
Q

What clinical subtypes of JIA have recently been identified?

A
  • Enthesopathy related arthritis
  • Juvenile psoriatic arthritis: oligo or spondylo with psoriasis or potential psoriasis
  • Others: unclassified under above criteria
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8
Q

How is pauciarticular JIA divided?

A
  • Type I 25%
  • Type II 15%
  • Type III 15%
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9
Q

What is the classical presentation of type I pauciarticular JIA?

A
  • Preschool girls
  • Limp rather than pain
  • Mainly LL joints
  • Knee>ankle>hand or elbow (hip very rare)
  • Chronic uveitis
  • Irregular iris due to posterior synechiae
  • +ve ANA
  • Can be asymptomatic
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10
Q

How is pauciarticular JIA defined?

A

4 or less joints

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11
Q

What is the classical presentation of type II pauciarticular JIA?

A
  • Primary aged boys (8-9)
  • Limp due to LL affection
  • Mainly LL joints: knee and ankle
  • Hips may be affected early with rapid damage requiring THR
  • Enthesitis
  • Can develop AS or spondyloarthritis
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12
Q

Which individuals with type II pauciarticular JIA are categoreised as juvenile ankylosing spondylitis?

A

Those with HLA-B27 + back involvement

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13
Q

What is the classical presentation of type III pauciarticular JIA?

A
  • Girls during childhood
  • Asymmetric UL and LL arthritis
  • Dactylitis
  • Chronic iridocyclitis
  • FH of psoriasis
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14
Q

What can happen to those with extended oligoarthritis?

A

30% of these presenting with pauciarticular JIA can go on to more severe polyarticular course.

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15
Q

How is polyarticular JIA defined?

A

5 or more joints

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16
Q

What is the classical presentation of RF- polyarticular JIA?

A
  • Girls of any age (often early)
  • Constitutional manifestations (low grade fever, malaise)
  • Hepato-splenomegaly
  • Mild anemia
  • Growth abnormalities
  • Symmetric large and small joints affection: knees, wrists, ankles, MCPs, PIPs, neck
  • Iridocyclitis rare
17
Q

What is the classical presentation of RF+ polyarticular JIA?

A

-Girls in later childhood (including teens)
-Constitutional manifestations (low grade fever, malaise, weight loss)
-Anaemia
-Nodules
Iridocyclitis is rare
-Similar to adult RA

18
Q

What can be seen on x-ray of RF+ polyarticular JIA?

A

Erosions which occur early

19
Q

What can complicate RF+ polyarticular JIA?

A
  • Sjorgen’s
  • Felty
  • Vasculitis
  • AR
  • Pulmonary fibrosis
  • AAS
  • CTS
20
Q

How is systemic onset JIA defined?

A

Extra-articular features which start early and disappear after 2-5 years

21
Q

What is another name for systemic onset JIA ?

A

Still’s disease

22
Q

What is the classical presentation of Still’s disease?

A
  • F:M 1.5:1
  • Occurs throughout childhood (4-6 years)
  • Joints affected include wrists, knees, ankles, cervical spine, hips and TMJ
  • Evanescent salmon red eruption on trunk and thighs accompanised by fever. Exhibits Koebner’s phenomenon
23
Q

What extra-articular features can be present in Still’s disease?

A
  • Pleural effusion
  • Pulmonary fibrosis
  • Polyserositis
  • Pericarditis
  • Tamponade and myocarditis
  • Generalised non-tender lymphadenopathy
  • Hepatosplenomegaly
  • Abdominal pain +- transaminases
24
Q

Describe the associated fever of Still’s disease.

A
  • Rise to 39.5 C 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 +/- chills but looks normal when fever goes away
25
Q

What is the 1st line therapy for JIA?

A
  • Simple painkillers

- NSAIDs

26
Q

What is the 2nd line therapy for JIA?

A

If no response to NSAIDs/ joint (steroid) injections.
(Rarely needed in oligoarticular JIA)
-Methotrexate (pharmocokinetics is age related).
-Anti-TNF Rx. (all 3): In methotrexate failure.
-IL-1 R-antagonist (Anakinra) in refractory systemic arthritis.
-IL-6 antagonist (Tocilizumab) for refractory systemic disease.

27
Q

Why is there limited indications for systemic steroids?

A

Due to their serious side effects

28
Q

What are systemic steroids used in?

A
  • Systemic JIA (control pain and fever)
  • Serious disease complications with any subtype e.g. periocordial effusion, tamponade, vasculitis, severe auto-immune anemia, severe aye disease
  • As a bridge between DMARDs
  • Children undergoing surgery
29
Q

What are the risks of systemic steroid use?

A
  • Osteoporosis
  • Infections
  • Growth abnormalities
30
Q

When are local steroids used?

A
  • Intra-articular mainly in Oligo-articular JIA

- Eye disease (ANA +ve oligo-articular disease

31
Q

What non-pharmacological therapy can be used?

A

Rehabilitation

  • Physiotherapy
  • Occupational therapy
32
Q

What surgical treatment is there?

A
  • Synovectomy

- Reconstructive/joint replacement surgery

Systems: MSK AB (43 decks)

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