Juvenile Idiopathic Arthritis

Question 1

What is juvenile idiopathic arthritis?

Answer 1

Is a group of systemic inflammatory disorders affecting children < 16 years

Question 2

What is the aetiology of JIA?

Answer 2

Aetiology:

• Autoimmune disease with multifactorial pathogenesis

Question 3

What does JIA stand for?

Answer 3

Juvenile idiopathic arthritis

Question 4

What is the prevalence of JIA?

Answer 4

1/1000 children <16 years

Question 5

What is the critieria for diagnosis of JIA?

Answer 5

• Age of onset
  
  • <16 years
• Duration of disease
  
  • >6 weeks
• Presence of arthritis
  
  • Joint swelling or 2 of the following
    
    • Painful or limited joint motion
    • Tenderness
    • Warmth

Question 6

What are the different clinical subtypes of JIA?

Answer 6

Pauciarticular (most prevalent)

Polyarticular

Systemic onset

Question 7

What is the most common clinical subtype of JIA?

Answer 7

Pauciarticular

Question 8

How many joints does pauciarticular JIA affect?

Answer 8

4 or less

Question 9

What are the different types of pauciarticular JIA?

Answer 9

• Type 1
  
  • Most prevalent type
  • Age < 5 years
  • Girls:boys 8:1
  • Presentation is limp rather than pain and stiffness, chronic uveitis, irregular iris
  • Knee > ankle > hand or elbow
• Type 2
  
  • Age after 8-9
  • Girls:boys 1:7
  • Presentation – lower limp joint affected (hip can be affected), acute iridocyclitis
• Type 3
  
  • Any age
  • Girls:boys 4:1
  • Presentation – constitutional rare, asymmetric upper limb or lower limb, dactylitis, chronic iridocyclitis
  • Can be associated with psoriasis (family history)
• Extended oligoarthritis
  
  • Present in pauciarticular way (<4 joints) but then go onto develop polyarticular arthritis

Question 10

What is the most prevalent type of pauciarticular JIA?

Answer 10

Type 1 pauciarticular JIA

Question 11

Describe the epidemiology of type 1 pauciarticular JIA in terms of sex and age?

Answer 11

• Age < 5 years
• Girls:boys 8:1

Question 12

Describe the epidemiology of type 2 pauciarticular JIA in terms of sex and age?

Answer 12

• Age after 8-9
• Girls:boys 1:7

Question 13

Describe the epidemiology of type 3 pauciarticular JIA in terms of sex and age?

Answer 13

• Any age
• Girls:boys 4:1

Question 14

What is the presentation of type 1 pauciarticular JIA?

Answer 14

• Presentation is limp rather than pain and stiffness, chronic uveitis, irregular iris

Question 15

What is the presentation of type 2 pauciarticular JIA?

Answer 15

• Presentation – lower limp joint affected (hip can be affected), acute iridocyclitis

Question 16

What is the presentation of type 3 pauciarticular JIA?

Answer 16

• Presentation – constitutional rare, asymmetric upper limb or lower limb, dactylitis, chronic iridocyclitis

Question 17

What type of pauiarticular JIA can be associated with psoriasis?

Answer 17

Type 3

Question 18

Where does type 1 pauciarticular JIA most commonly affect?

Answer 18

• Knee > ankle > hand or elbow

Question 19

What is extended oligoarthritis?

Answer 19

• Present in pauciarticular way (<4 joints) but then go onto develop polyarticular arthritis

Question 20

How many joints does polyarticular JIA affect?

Answer 20

5 or more

Question 21

What are the clinical subtypes of polyarticular JIA?

Answer 21

• Divided into rheumatoid factor + and –

Question 22

Describe the epidemiology of polyarticular -ve JIA in terms of sex and age?

Answer 22

• Any age
• Girls:boys 9:1

Question 23

Describe the epidemiology of polyarticular +ve JIA in terms of sex and age?

Answer 23

• Late childhood
• Girls:boys 7:1

Question 24

What is the presentation of polyarticular -ve JIA?

Answer 24

• Presentation – constitutional manifestations (fever, malaise), hepato-splenomegaly, anaemia, restriced growth, symmetric arthritis on large and small joints (knees, wrists, ankles, MCPs, PIPs, neck), uveitis rare

Question 25

What is the presentation of polyarticular +ve JIA?

Answer 25

* Presentation – constitutional manifestations, anaemia, nodules, erosions in x-ray, uveitis rare

Question 26

What is systemic onset JIA known as?

Answer 26

STILL's disease

Question 27

Describe the epidemiology of systemic onset JIA in terms of sex and age?

Answer 27

* Any age * Girls:boys 1.5:1

Question 28

What is the characteristic presentation of systemic onset JIA?

Answer 28

* Characteristic presentation is fever that rises daily then returns back to normal after 2 weeks with no other obvious cause, rash described as salmon red eruption on trunk and thighs, lymphadenopathy, hepatosplenomegaly with abdominal pain, serositis, lung involvement rare

Question 29

Is the arthritis in systemic onset JIA usually pauciarticular or polyarticular?

Answer 29

Polyarthritis

Question 30

What is uveitis?

Answer 30

Inflammation of the middle layer of eyeball, which consists of iris, ciliary, body and choroid

Question 31

In what clinical subtype of JIA is uveitis most common?

Answer 31

Question 32

What should every child with arthritis be screened for?

Answer 32

Eye problems (uveitis)

Question 33

What are potential complications of uveitis?

Answer 33

Is a cause of potential blindness, other complications includes: * Posterior synechiae * Cataracts * Band keratopathy

Question 34

What is the treatment of uveitis?

Answer 34

* Steroids * First topical, sometimes systemic required * Mydriatic and cycloplegic agents * Methotrexate * MMF * Ciclosporin * Anti-TNF

Question 35

Describe the management of JIA?

Answer 35

* First line therapy * Simple pain killers * NSAIDs * Second line therapy * If no response to NSAIDs/joint steroid injection * 1) Methotrexate * 2) Anti-TNF * 3) IL-1 R-antagonist in refractory systemic arthritis * 4) IL-6 antagonist for refractory systemic disease * Rehabilitation * Physiotherapy * Occupational therapy * Surgical treatment * Synovectomy * Reconstructive/joint replacement surgery

Question 36

What is the first line therapy for JIA?

Answer 36

* First line therapy * Simple pain killers * NSAIDs

Question 37

What is the second line therapy for JIA?

Answer 37

* Second line therapy * If no response to NSAIDs/joint steroid injection * 1) Methotrexate * 2) Anti-TNF * 3) IL-1 R-antagonist in refractory systemic arthritis * 4) IL-6 antagonist for refractory systemic disease

Question 38

Why do systemic steroids have limited indications in JIA?

Answer 38

Due to serious side effects

Question 39

What are indications for systemic steroids in JIA?

Answer 39

* Systemic JIA to control pain and fever * Serious disease complications with any subtype * As a bridge between DMARDs

Question 40

What kinds of growth failure can occur in JIA?

Answer 40

* Localised growth failure * Leg length discrepancies * Shortening of fingers, hands, forearms, toes, feet * Micrognathia (poor growth of mandible) * Implications for tooth development and appearance * Generalised growth failure * Related to severe systemic disease * Short stature * Delayed puberty

Question 41

What is micrognathia?

Answer 41

* Micrognathia (poor growth of mandible) * Implications for tooth development and appearance