Ch. 17 - Polyarticular JIA

Question 1

How to determine RF positivity

Answer 1

Detection of RF on 2 occasions at least 3 months apart

Question 2

RF+ vs RF- Polyarthritis: More common

Answer 2

RF- (85%)

Question 3

T/F: Onset age of RF- polyarthritis reflects a biphasic trend

Answer 3

T, peaking at 1-3y then in later childhood and adolescence

Question 4

T/F: Younger onset polyarticular RF- JIA are more likely to be ANA (+)

Answer 4

T

Question 5

T/F: Younger onset polyarticular RF- JIA is correlated with a poorer outcome

Answer 5

T

Question 6

Acute vs insidious: Onset of RF- polyarticular JIA

Answer 6

Insidious

Question 7

T/F: In RF- polyarthritis, joint disease predominates and extraarticular features are infrequent

Answer 7

T

Question 8

T/F: In RF- polyarticular JIA, joints are generally red and tender

Answer 8

F, warm but generally NOT red and tender

Question 9

MC joints affected by RF- polyartciular JIA

Answer 9

Knees, ankles, and wrists

Question 10

T/F: DIPs are commonly affected at onset in RF- poly JIA

Answer 10

F

Question 11

RF- vs RF+ poly JIA: TMJ involvement

Answer 11

RF-

Question 12

RF- vs RF+ poly JIA: Earlier onset

Answer 12

RF-

Question 13

T/F: TMJ arthritis can be present without overt signs and symptoms

Answer 13

T

Question 14

T/F: Cervical spine involvement is NOT common in early RF- poly JIA

Answer 14

T

Question 15

RF- vs RF+ poly JIA: More joints affected

Answer 15

RF+

Question 16

RF- vs RF+ poly JIA: More symmetric joint involvement

Answer 16

RF+

Question 17

RF- vs RF+ poly JIA: Radiological signs of hip involvement

Answer 17

RF-

Question 18

Involvement of these joints at 1st presentation has been suggested as predictor of progression to polyarthritis in those 1st presenting with oligoarthritis

Answer 18

Wrist and ankle

Question 19

Uncommon subset of RF- poly JIA that has also been suggested to be a forme fruste of scleroderma

Answer 19

Dry synovitis (polyarthropathy with minimal or absent clinical signs of joint effusion)

Question 20

T/F: Fever seldom occurs in RF- poly JIA, and if present is typically low grade

Answer 20

T

Question 21

Growth parameter measured in the assessment of growth of JIA patients

Answer 21

Height for age z score

Question 22

T/F: Height for age of JIA patient tend to return to normal

Answer 22

T

Question 23

RF+ vs RF- poly JIA: Negative deviation in ht for age is less marked and less prolonged

Answer 23

RF-

Question 24

T/F: Growth velocity in JIA tend to correlate with disease severity and activity and with the number of involved joints

Answer 24

T

Question 25

T/F: RF- poly JIA is commonly associated with overt CV pathology

Answer 25

F

Question 26

T/F: Compared to JIA, arthritis of SLE is nonerosive

Answer 26

T

Question 27

Ddx for JIA presenting with joint contractures of the small joints of the hands BUT without associated signs of intraarticular swelling

Answer 27

Scleroderma

Question 28

Ddx for JIA that is infectious in origin and is characterized by an early migratory phase

Answer 28

N. gonorrheae arthritis

Question 29

T/F: Compared to JIA, ARF arthritis is non-erosive

Answer 29

T

Question 30

T/F: Joint involvement in malignancy tends to be polyarticular

Answer 30

F, oligoarticular

Question 31

Ddx of RF- poly JIA in children at risk for nutritional deficiencies, including those with autism and other developmental disorders

Answer 31

Scurvy

Question 32

T/F: Patients with RF- poly JIA typically have very elevated markers of inflammation

Answer 32

F, moderately elevated only

Question 33

What do RFs bind to in the immune system

Answer 33

CH2 and CH3 domains of the Fc portion of IgG

Question 34

Agglutination assays for the detection of RF typically detect what Ig subtype

Answer 34

Pentameric IgM RF

Question 35

More sensitive assay compared to agglutination assay for detection of RF

Answer 35

EIA

Question 36

Done so that hidden RFs can generate a response in agglutination assays

Answer 36

Acid elution to separate them from IgG

Question 37

Proportion of RF- poly JIA that present with (+) ANA

Answer 37

~50%

Question 38

Characteristics of JIA subgroups with (+) ANA

Answer 38

Early onset, female predominance, asymmetric arthritis, risk for uveitis

Question 39

Cells that predominate in synovial fluid of patients with RF- poly JIA

Answer 39

PMN

Question 40

Oligo vs poly JIA: Greater vascularity of the synovium on pathologic sample

Answer 40

Poly

Question 41

Mainstays of treatment in RF- poly JIA

Answer 41

Early and judicious use of pharmacotherapy PT and OT Promotion of healthy lifestyles Optimal nutrition Physical activity Reduction of stress

Question 42

Medical management of RF- poly JIA

Answer 42

NSAIDs > MTX > Leflunomide > Anti-TNF > Anti-IL-6

Question 43

Unfavorable prognostic factors in RF- poly JIA that warrants more aggressive early treatment

Answer 43

1. Hip and cervical spine involvement 2. Radiographic evidence of joint space narrowing and/or bone erosions 3. Presence of ACPA

Question 44

Indicators of moderate to severe RF- poly JIA that warrants prompt aggressive therapy

Answer 44

1. Number of active joints 2. Levels of inflammatory markers 3. Poor physician and patient/parent global assessments

Question 45

RF- JIA therapy: Failure of NSAIDs to work within this time frame warrants prompt ADDITION of MTX

Answer 45

6 weeks

Question 46

RF- JIA therapy: MTX is usually given by mouth initially at a dose of

Answer 46

10-15mg/BSA/week

Question 47

RF- JIA therapy: In the absence of response to oral MTX at 10-15mg/BSA/week, dosing can be modified to

Answer 47

15-20mg/BSA/week preferably given SQ

Question 48

RF- JIA therapy: T/F Response to MTX is usually excellent

Answer 48

T

Question 49

T/F: RF status in poly JIA correlates with responsiveness to anti-TNF

Answer 49

F

Question 50

RF- JIA therapy: Patients with moderate to high disease activity who fail to respond to MTX or Leflunomide within this time frame warrants consideration of anti-TNF therapy

Answer 50

6 months

Question 51

RF- JIA therapy: Patients with low disease activity who fail to respond to MTX or Leflunomide within this time frame warrants consideration of anti-TNF therapy

Answer 51

6 months

Question 52

RF- JIA therapy: Drug that targets IL-6

Answer 52

Tocilizumab

Question 53

RF- JIA therapy: Have limited use in systemic therapy but can be a bridging agent until DMARDs become effective

Answer 53

Glucocorticoid

Question 54

RF- JIA therapy: When should focused PT be instituted

Answer 54

As soon as inflammation subsides sufficiently to facilitate the child’s cooperation

Question 55

Children with RF- poly JIA who have not remitted by this age are likely to have active arthritis into their 20s or early 30s

Answer 55

16y

Question 56

T/F: RF- poly JIA is associated with substantial morbidity in most affected children

Answer 56

T