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Year 3: Block 1: MSK system > Juvenile idiopathic arthritis > Flashcards

Juvenile idiopathic arthritis Flashcards

1
Q

What is JIA?

What can it cause in young people as well?

A

Group of systemic inflammatory disorders affecting children below age of 16 years.
The most commonly diagnosed Rheumatic disease in children.
An important cause of disability and blindness.

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2
Q

history and pathogenesis

  • what may affect the immune response?
A

auto immune
- Etiopathogenesis is multi-factorial and different from that of adult RA.

  • Strong subset-specific genetic markers may affect immune response.
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3
Q

Most common cause for physical disability ?

A

JIA

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4
Q

Etiology and Pathogenesis
Age of onset?
Duration of disease?
Presence of arthritis?

A

<16 years

> 6 weeks

Joint swelling or 2 of the following:
Painful or limited joint motion
Tenderness
Warmth

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5
Q

Clinical subtypes of JIA

-most common subtype?

A

Pauciarticula

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6
Q

most common subtype divided into

A

Rheumatoid factor positive and negative

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7
Q

all subtypes are greater in females apart from what one?

A

enthesitis related

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8
Q

Pauciarticular JIA (4 or less joints) - describe the 3 types - type 1

A

Majority of pauci (25%)
Age: before 5 years, peak 1-3 years
Girls: boys = 8:1

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9
Q

Presentation of children with arthritis

A
  • limp rather than pain

No constitutional manifestations

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10
Q

Pauciarticular JIA (4 or less joints) - what joints are involvement? - type 1

A

Mainly LL joints

Knee> ankle> hand or elbow (hip very rare).

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11
Q

What is positive in Pauciarticular JIA - type 1

  • what is common that can be asymptomatic?
A

ve ANA in 40-75%

Chronic uveitis in 20% of cases (95% if female < 2years old)
Asymptomatic in 50%
Irregular iris due to posterior synechiae (get eyes looked at)

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12
Q

Pauciarticular JIA - type 2

  • what joints are affected?
A

More common in boys
can get acute episodes

Mainly LL joints: knee, ankle.
Hip can be affected early with rapid damage requiring THR early in life + enthesitis + many have sacroilliac joints and may evolve AS or spondyloarthritis
20% difficult to classify to particular spondyloarthropathy group.

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13
Q

Pauciarticular JIA - type 2 - those who lack back problems called?

slightly older patients

A

into sero-ve enthesopathy arthropathy (SEA) syndrome who less frequently inherit HLA-B27

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14
Q

Those with HLA-B27 + inflammatory back symptoms can be termed as having?

A

Juvenile Ankylosing spondylitis.

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15
Q

Pauciarticular JIA - type 3 - features - what can it be associated with?

younger patients

A

constitutional rare
asymmetric UL and LL arthritis
dactylitis.

Arthritis can be very destructive
Family history of psoriasis in 40%
+/- nail pitting
These patients may develop psoriasis later in life

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16
Q

Pauciarticular JIA - type 3 - what can they develop

A

Chronic iridocyclitis in 10-20%.

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17
Q

Pauciarticular JIA - what can they go on to develop?

A

Extended oligoarthritis:

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

18
Q

WHta is dactylitis?

A

nail bed
linked with psoriasis
- fungal infections

19
Q

Polyarticular JIA (5 or more joints) - rheumatoid factor negative?

A

girls more than boys

- early age

20
Q

Polyarticular JIA (5 or more joints) - rheumatoid factor negative - presentation

A

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

Symmetric large and small joints affection: knees, wrists, ankles, MCPs, PIPs, neck

21
Q

What is rare in Polyarticular JIA negative

A

Iridocyclitis rare.

22
Q

Polyarticular JIA (5 or more joints) - rheumatoid factor positive - presentation

A

later years

Presentation:
Constitutional manifestations (low grade fever, malaise, weight loss)
Anemia
Nodules.

23
Q

Systemic complications of Polyarticular JIA (5 or more joints) - rheumatoid factor positive

A

Can be 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

24
Q

Systemic onset JIA - what defines ?

A

Extra-articular features define the disease

25
Q

Systemic onset JIA - presentation

A

fever that rises daily and goes back to normal - presentationt for 2 weeks

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

26
Q

Systemic onset JIA - what is there a typical clinical sign of? GIVE features

A 
RASH 
90%
Evanescent salmon red eruption
On trunk and thighs 
Accompanies fever
Can be brought by scratching (+ve Koebner’s phenomenon).
27
Q

Systemic features of Systemic onset JIA

A 
Generalized lymphadenopathy
Hepatosplenomegaly
50-75%
Abdominal pain
\+/- transaminases
- Polyserositis
Pericarditis in 36%
Tamponade and myocarditis rare

Rare
Pleural effusion
Pulmonary fibrosis

28
Q

Systemic onset JIA - what is the arthritis like
when does it present?

joints affected?

A

poly arthritis

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

29
Q

Important clinical sign

A

loss of hyper mobility

30
Q

What should you detect in children with arthritis?

A

jaw movement

  • jaw ma be pulled towards the affected side
31
Q

When are there higher levers of uveitis (2)

A

oligo-articular and extended oligo groups

  • related to ANA positivity in EOIJA
32
Q

Uveitis complications (3)

lots of patients will develop it

A

potential blindness
- can affect all groups

early in the disease course

  • posterior synechiae - iris sticks onto the Lens
  • cataract
  • band keratopathy
  • glaucoma
  • eye surgery
  • Sicca syndrome - dry eyes
33
Q

Treatment and management of uveitis

A

screening!!! - all age groups

treatment:
- steroids : typical: intraoccular

  • mydriatic agents/cycloplegic agents
  • Methotrexate
  • MMF
  • Ciclosporin
    Anti-TNF
34
Q

1st line therapy?

some features you must consider with NSAIDS

A 
Simple pain killers
NSAIDs:
Difference between adults and children half life
Can control disease
Doses
Same compounds only
35
Q

2nd line therapy in children? what drugs do you use (1-4)

when are these drugs used?
when are these drugs rarely needed?

A

Methotrexate (pharmocokinetics is age related).

(2) Anti-TNF Rx. (all 3): In methotrexate failure.
(3) IL-1 R-antagonist (Anakinra) in refractory systemic arthritis.
(4) IL-6 antagonist (Tocilizumab) for refractory systemic disease

If no response to NSAIDs/ joint (steroid) injections.
- Rarely needed in oligoarticular JIA

36
Q

Systemic steroids

When are they used?

A

Limited indications due to serious side effects

  • 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
37
Q

What are the risks of systemic steroids?

A

of osteoporosis, infections, growth abnormalities

38
Q

Local steroids are used mainly in?

how are they used?

A
  • Oligo-articular JIA

- TOPICALLY for the Eye disease (ANA +ve oligo-articular disease).

39
Q

Surgical treatment (2)

A
  • Synovectomy

- Reconstructive / joint replacement surgery

40
Q

Growth failure in JIA? the 2 types and features of both

A

localised - leg length discrepancies, shortening of fingers, hands, forearms, toes and feet, JAW - Mircrognathia (poor growth of the mandible) - receding chin

Generalised

  • related to severe systemic
  • short stature
  • delayed puberty
  • systemic steroids
41
Q

Mircrognathia - often need (3)

A

orthodontic intervention
receding chin

  • TMJ resistant = vigilant in MRI scanning, intra- ocular steroids to prevent growth failure
42
Q

What is not a diagnostic factor?

A

ANA - important to do it - helps to categorise their arthritis as much as possible

Year 3: Block 1: MSK system (22 decks)

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