CHAPTER 11
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Juvenile Idiopathic Arthritis
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TABLE 11-1
Comparison of JRA, JCA, and JIA Classifications
JRA
JCA
JIA
Committee
ACR
EULAR
ILAR
Age at onset
<
16 yr
<
16 yr
<
16 yr
Disease duration
>
6 wk
>
3 mo
>
6 wk
Onset types
Pauciarticular
Polyarticular
Systemic
Pauciarticular
Polyarticular RF-negative
Juvenile rheumatoid arthritis
Systemic
Juvenile psoriatic arthritis
Juvenile ankylosing spondylitis
Oligoarticular, persistent
Oligoarticular, extended
Polyarticular RF-negative
Polyarticular RF-positive
Systemic
Psoriatic arthritis
Enthesitis-related arthritis
Exclusions
Juvenile psoriatic arthritis
Juvenile ankylosing spondylitis
Inflammatory bowel disease
Other forms of juvenile arthritis
Other forms of juvenile arthritis
Other forms of juvenile arthritis
RF, rheumatoid factor.
JIA Subtype
Exclusions
a
Inclusion Criteria
b
Oligoarthritis
1–5
Persistent
≤
4 joints during disease course
Extended
>
4 joints after the first 6 mo
Polyarthritis RF-negative
1–5
Arthritis affecting
≥
5 joints during the first 6 mo
Polyarthritis RF-positive
1–3, 5
Arthritis affecting
≥
5 joints during the first 6 mo,
plus RF positivity on two occasions more than 3 mo apart
Systemic
1–4
Arthritis with or preceded by daily fever of at least 2 weeks’ duration,
accompanied by one or more of the following:
Evanescent, nonfixed erythematous rash
Generalized adenopathy
Hepatomegaly or splenomegaly
Serositis
Psoriatic
2–5
Arthritis and psoriasis, or arthritis and at least two of the following:
a. Dactylitis
b. Nail abnormalities (pitting or onycholysis)
c. Family history of psoriasis in a first-degree relative
Enthesitis-related
1, 4, 5
Arthritis and enthesitis, or arthritis or enthesitis with at least two of the
following:
1. SI joint tenderness and/or inflammatory spinal pain
2. Presence of HLA-B27
3. Family history of HLA-B27–associated disease in a first-degree relative
4. Onset of arthritis in a male after the age of 6 yr
Undifferentiated
Children with arthritis of unknown cause that persists
≥
6 wk
Does not fulfill criteria for any of the other categories
Fulfills criteria for
≥
1 of the other categories
a
Exclusions: 1, psoriasis in the patient or a first-degree relative; 2, arthritis in an HLA-B27 positive male beginning after the sixth birthday; 3, ankylosing spondylitis,
enthesitis-related arthritis, sacroiliitis with IBD, Reiter syndrome, or acute anterior uveitis in a first-degree relative; 4, IgM RF on at least two occasions more than 3 mo
apart; 5, presence of systemic JIA.
b
Inclusion criteria for all subtypes: 1, age at onset
<
16 yr; 2, arthritis in one or more joints; 3, duration of disease is at least 6 wk.
From Petty RE, et al. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol
2004;31(2):390–392.
TABLE 11-2
Criteria for Classification of JIA
