Juvenile Idiopathic Arthritis Flashcards

1
Q

Define Juvenile Idiopathic Arthritis

A
  • Group of systemic inflammatory disorders affecting children <16 y/o
  • The most commonly diagnoses rheumatic disease in children
  • An important cause of disability blindness
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2
Q

Causes of JIA

A
  • Autoimmune disease
  • Multifactorial + different from adult RA
  • Genetic, environmental, immunological
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3
Q

Criteria for diagnosis of JIA

A

-Age of onset <16 y/o
->6 week duration of disease
-Presence of arthritis
Joint swelling OR 2 from the following
Painful/limited joint motion
Tenderness
Warmth

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4
Q

Subtypes of JIA

A

-Pauciarticular
-Polyarticular
-Systemic onset
-

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5
Q

Most common subtype of JIA and its subtypes

A
  • Pauciarticular

- Type 1, 2, 3

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6
Q

Presentation of pauciarticular JIA

A
  • <5y/o (peak age 1-3)
  • Girls:boys = 8:1
  • Limp rather than pain
  • Mainly lower limb joints
  • Knee>Ankle>Hand/Elbow (hip v rare)
  • +ve ANA in 40-75%
  • Irregular iris due to posterior synechiae (iris adheres to lens)
  • Chronic uveitis in 20%
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7
Q

What presenting symptom is increased in rate in girls <2y/o

A

Chronic uveitis (95% from 20%)

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8
Q

Presentation of type 2 pauciarticular JIA

A
  • > 8/9y/o
  • Boys:Girls = 7:1
  • Limp due to lower limb affection
  • Mainly knee + ankle
  • Hip can be affected with rapid damage (requiring THR)
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9
Q

In type 2 pauciarticular JIA what may the hip damage lead to

A
  • THR
  • Enthesitis
  • AS or spondyloarthritis
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10
Q

Presentation of type 3 pauciarticular JIA

A
  • Any age during childhood
  • Girls:boys = 4:1
  • Asymmetric upper + lower limb arthritis
  • Dactylitis
  • Can be v destructive
  • FHx of psoriasis in 40%(child may develop psoriasis later in life)
  • +/-nail pitting
  • Chronic iridocyclitis
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11
Q

How many joints need to be affected to classify JIA as polyarticular JIA

A

5 or more

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12
Q

How many joints need to be affected to classify JIA as pauciarticular JIA

A

4 or less

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13
Q

Most common type of JIA

A

Pauciarticular arthritis

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14
Q

Presentation of polyarticular JIA when RF is -ve

A

-Any age, often early
-Girls:boys = 9:1
-Systemic features (low grade fever + malaise)
-Hepato-splenomegaly
-Mild anaemia
-Growth abnormalities
-Symmetric large + small joints affection (knees, wrists, ankles, MCPs, PIPs, neck)
-Iridocyclitis is rare
-

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15
Q

Presentation of polyarticular JIA when RF is +ve

A
  • Late childhood (12-16y/o)
  • Girls:boys = 7:1
  • Systemic features (low grade fever, malaise, weight loss)
  • Anaemia
  • Nodules
  • Similar to adult RA
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16
Q

What may be seen on X-rays of RF +ve polyarticular JIA

A

Erosions may occur early

17
Q

What may complicate RF +ve polyarticular JIA

A

-Vasculitis
-AR
-Pulmonary fibrosis
and others

18
Q

What is rare in both RF +ve and -ve polyarticular JIA

A

Iridocyclitis

19
Q

Least common but most serious subtype of JIA

A

Systemic onset JIA

20
Q

What defines systemic onset JIA

A

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

21
Q

Systemic onset JIA AKA

A

Still’s Disease

22
Q

Presentation of systemic onset JIA

A
  • Throughout childhood (4-6 years)
  • Girls:boys = 1.5:1
  • Fever
  • Rash
  • Non-tender lymphadenopathy
  • Abdo pain
  • Hepato-splenomegaly
  • Polyserositis, pericarditis
  • Arthritis in knees, wrists, ankles, C-spine, hips and TMJ
23
Q

Describe the fever seen in systemic onset JIA

A
  • Rise to 39.5 daily for at least 2 weeks
  • Late afternoon/evening + returns to normal/subnormal in morning
  • Child appears toxic with fever +/- chills but looks well when fever goes away
24
Q

Describe the rash seen in systemic onset JIA

A
  • Evanescent salmon red eruption
  • On trunk + thighs
  • Accompanies fever
  • Can be brought by scratching (+ve Koebner’s phenomenon)
  • Occurs in 90%
25
Q

What are rare CVS and resp. presentations of systemic onset JIA

A
  • Tamponade
  • Myocarditis
  • Pulmonary fibrosis
  • Pleural effusion
26
Q

When is the arthritis seen in systemic onset JIA

A

Within first 3-12 months of onset of fever

27
Q

Investigations for systemic onset JIA

A
  • ESR (very high)

- ASO(elevated with no evidence of recent strep infection)

28
Q

Investigations for polyarticular JIA

A

-RF

29
Q

Investigations for pauciarticular JIA

A

-ANA (antinuclear antibodies)

30
Q

Rx of JIA

A

-Simple analgesics
-IA NSAIDs/steroids
If no response
-Methotrexate
-Anti-TNF (if methotrexate fails)

Physio + Occ therapy

31
Q

Indication for systemic steroids in JIA

A
  • Systemic onset JIA
  • Serious complication with any disease (Pericardial effusion, tamponade, vasculitis)
  • As a bridge between DMARDs
  • Children undergoing surgery
32
Q

Risk of using systemic steroids

A
  • Osteoporosis
  • Infections
  • Growth abnormalities
33
Q

Surgical Rx for JIA

A
  • Synovectomy

- Reconstructive/joint replacement