JIA

Question 1

how common is JIA

Answer 1

• most common rheumatologic disease in children
• 2-23/100 000

Question 2

causes of JIA

Answer 2

• not completely understood
• some genetic susceptibility
• immune response
• pro-inflammatory markers - TNF, IL-1, 2
• antibodies

Question 3

features of JIA

Answer 3

• arthritis for ≥6wks
• morning stiffness or gelling
• irritability/refusal to walk in toddlers
• school absence or limited ability to participate in physical activity
• rash/fever
• fatigue
• poor appetite/weight loss
• delayed puberty

Question 4

DDx for JIA

Answer 4

• septic arthritis
• osteomyelitis
• transient synovitis
• malignancies e.g. lymphoma, neuroblastoma, bone tumours
• recurrent haemoarthrosis
• vascular abnormalities
• trauma

Question 5

signs of JIA

Answer 5

• swelling: periarticular soft tissue oedema, intraarticular effusion, hypertrophy of synovial membrane
• tenosynovitis (swollen tendons)
• pain
• joint held in position of max comfort
• ROM limited at extremes

Question 6

classifications of JIA

Answer 6

Question 7

exclusions for each category in order to avoid cross over

Answer 7

• psoriasis or hx of in the pt or 1st degree relative
• arthritis in HLA-B27+ve male beginning >6y/o
• ankylosing spondylitis, enthesitis related arthritis, sacroiliitis w/ IBD or acute anterior uveitis or a hx of one of these in 1st degree relative
• presence of IgM rheumatoid factor and at least 2 occasions at least 3mths apart
• presence of systemic JIA
• RF

Question 8

JIA pattern of onset

Answer 8

4 or fewer joints/large joints no symmetry

→ oligoarthritic or pauciarticular (40-60%)

→ early

Question 9

polyarticular JIA patterns of onset

Answer 9

• can be acute but is mostly insidious
• large fast growing joints are mostly affected
• TMJ joint injury is common leading to limited bite and micrognathia
• systemic manifestations rare: fever, slight hepatosplenomegaly, lymphadenopathy, pericarditis, chronic uveitis

Question 10

enthesitis related JIA features

Answer 10

inflammation of enthesis along with arthritis

plus 2 out of:

• inflammatory spinal pain
• sacroiliac joint tenderness
• FHx of enthesitis related JIA
• onset of poly/oligoarthritis in a boy >8y/o
• HLA B27+ve
• acute anterior uveitis

Question 11

enthesitis exam

Answer 11

Question 12

psoriatic JIA features

Answer 12

chronic arthritis and definite psoriasis is evident

HLA B27+ve

plus 2 out of:

• dactylitis: finger/toe inflamamtion
• onycholysis: nail pitting
• FHx psoriasis

Question 13

how common is systemic JIA

Answer 13

5-15% of JIA

Question 14

features of systemic JIA

Answer 14

• unwell
• arthritis
• intermittent fever >2wks
• salmon pink erythematous rash
• generalised lymphadenopathy
• serositis
• hepatomegaly/splenomegaly
• high inflammatory markers

Question 15

pGALS MSK screen questions

Answer 15

• do you/your child have any pain or stiffness in the joints, muscles or back
• do you/your child have any difficulty getting dressed w/o help
• do you/your child have any problem going up and down stairs

Question 16

investigations for JIA

Answer 16

labs

x-ray

US

MRI w/ contrast

Question 17

label the diagram

Answer 17

Question 18

normal joint vs OA and RA

Answer 18

Question 19

goals of treatment

Answer 19

• pharmacologic management: NSAIDs, DMARDs, biologic agents, intra-articular/oral steroids
• psychosocial factors: incl counselling for pts and family
• school performance: school life adjustments, physical education adjustments
• nutrition: anaemia, generalised osteoporosis
• PT: relieve pain, address ROM, muscle strength, ADLs, conditioning exercises
• OT: joint protection, programme to relieve pain, ROM, ADLs

Question 20

intra-articular steroids - effectiveness

Answer 20

• highly effective
• remission >6mth 84%
• greater success in oligoarticular JIA
• safe and effective
• no long term SEs

Question 21

DMARDs - effectiveness

Answer 21

• methotrexate
• poor response to IAS in oligo JIA
• commonly used
• should be used early for good outcome
• most given injectable (SC) form
• not many SEs
• blood monitoring

Question 22

biologics - when are they used and effectiveness

Answer 22

• failure to respond to DMARD
• anti-TNF agents commonly used
• good safety profile
• newer biologics available

Question 23

uveitis in JIA

Answer 23

• untreated can progress to chronic uveitis
• all children diagnosed w/ JIA undergo screening → early detection prevents complications
• uveitis more common in ANA+ve oligo JIA

Question 24

features of uveitis in JIA

Answer 24

• <5yrs
• rarely symptomatic
• red eyes, headache, reduced vision
• long term effects: cataracts, glaucoma, blindness

Question 25

treatment of uveitis

Answer 25

* slit lamp examination * all JIA pts seen within 6wks of diagnosis * high risk children * initially topical steroids to reduce inflammation, more severe need systemic * poor response to steroids → DMARD and biologics * early detection and treatment → good prognosis

Question 26

complications of JIA

Answer 26

poor growth localised growth disturbances micrognathia contractures ocular complications