Ch. 17 - Polyarticular JIA Flashcards
How to determine RF positivity
Detection of RF on 2 occasions at least 3 months apart
RF+ vs RF- Polyarthritis: More common
RF- (85%)
T/F: Onset age of RF- polyarthritis reflects a biphasic trend
T, peaking at 1-3y then in later childhood and adolescence
T/F: Younger onset polyarticular RF- JIA are more likely to be ANA (+)
T
T/F: Younger onset polyarticular RF- JIA is correlated with a poorer outcome
T
Acute vs insidious: Onset of RF- polyarticular JIA
Insidious
T/F: In RF- polyarthritis, joint disease predominates and extraarticular features are infrequent
T
T/F: In RF- polyarticular JIA, joints are generally red and tender
F, warm but generally NOT red and tender
MC joints affected by RF- polyartciular JIA
Knees, ankles, and wrists
T/F: DIPs are commonly affected at onset in RF- poly JIA
F
RF- vs RF+ poly JIA: TMJ involvement
RF-
RF- vs RF+ poly JIA: Earlier onset
RF-
T/F: TMJ arthritis can be present without overt signs and symptoms
T
T/F: Cervical spine involvement is NOT common in early RF- poly JIA
T
RF- vs RF+ poly JIA: More joints affected
RF+
RF- vs RF+ poly JIA: More symmetric joint involvement
RF+
RF- vs RF+ poly JIA: Radiological signs of hip involvement
RF-
Involvement of these joints at 1st presentation has been suggested as predictor of progression to polyarthritis in those 1st presenting with oligoarthritis
Wrist and ankle
Uncommon subset of RF- poly JIA that has also been suggested to be a forme fruste of scleroderma
Dry synovitis (polyarthropathy with minimal or absent clinical signs of joint effusion)
T/F: Fever seldom occurs in RF- poly JIA, and if present is typically low grade
T
Growth parameter measured in the assessment of growth of JIA patients
Height for age z score
T/F: Height for age of JIA patient tend to return to normal
T
RF+ vs RF- poly JIA: Negative deviation in ht for age is less marked and less prolonged
RF-
T/F: Growth velocity in JIA tend to correlate with disease severity and activity and with the number of involved joints
T
T/F: RF- poly JIA is commonly associated with overt CV pathology
F
T/F: Compared to JIA, arthritis of SLE is nonerosive
T
Ddx for JIA presenting with joint contractures of the small joints of the hands BUT without associated signs of intraarticular swelling
Scleroderma
Ddx for JIA that is infectious in origin and is characterized by an early migratory phase
N. gonorrheae arthritis
T/F: Compared to JIA, ARF arthritis is non-erosive
T
T/F: Joint involvement in malignancy tends to be polyarticular
F, oligoarticular
Ddx of RF- poly JIA in children at risk for nutritional deficiencies, including those with autism and other developmental disorders
Scurvy
T/F: Patients with RF- poly JIA typically have very elevated markers of inflammation
F, moderately elevated only
What do RFs bind to in the immune system
CH2 and CH3 domains of the Fc portion of IgG
Agglutination assays for the detection of RF typically detect what Ig subtype
Pentameric IgM RF
More sensitive assay compared to agglutination assay for detection of RF
EIA
Done so that hidden RFs can generate a response in agglutination assays
Acid elution to separate them from IgG
Proportion of RF- poly JIA that present with (+) ANA
~50%
Characteristics of JIA subgroups with (+) ANA
Early onset, female predominance, asymmetric arthritis, risk for uveitis
Cells that predominate in synovial fluid of patients with RF- poly JIA
PMN
Oligo vs poly JIA: Greater vascularity of the synovium on pathologic sample
Poly
Mainstays of treatment in RF- poly JIA
Early and judicious use of pharmacotherapy
PT and OT
Promotion of healthy lifestyles
Optimal nutrition
Physical activity
Reduction of stress
Medical management of RF- poly JIA
NSAIDs > MTX > Leflunomide > Anti-TNF > Anti-IL-6
Unfavorable prognostic factors in RF- poly JIA that warrants more aggressive early treatment
- Hip and cervical spine involvement
- Radiographic evidence of joint space narrowing and/or bone erosions
- Presence of ACPA
Indicators of moderate to severe RF- poly JIA that warrants prompt aggressive therapy
- Number of active joints
- Levels of inflammatory markers
- Poor physician and patient/parent global assessments
RF- JIA therapy: Failure of NSAIDs to work within this time frame warrants prompt ADDITION of MTX
6 weeks
RF- JIA therapy: MTX is usually given by mouth initially at a dose of
10-15mg/BSA/week
RF- JIA therapy: In the absence of response to oral MTX at 10-15mg/BSA/week, dosing can be modified to
15-20mg/BSA/week preferably given SQ
RF- JIA therapy: T/F Response to MTX is usually excellent
T
T/F: RF status in poly JIA correlates with responsiveness to anti-TNF
F
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
6 months
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
6 months
RF- JIA therapy: Drug that targets IL-6
Tocilizumab
RF- JIA therapy: Have limited use in systemic therapy but can be a bridging agent until DMARDs become effective
Glucocorticoid
RF- JIA therapy: When should focused PT be instituted
As soon as inflammation subsides sufficiently to facilitate the child’s cooperation
Children with RF- poly JIA who have not remitted by this age are likely to have active arthritis into their 20s or early 30s
16y
T/F: RF- poly JIA is associated with substantial morbidity in most affected children
T
