A. General. The JRA Criteria Committee has reviewed the initial criteria approved by the American Rheumatism Association in December 1971 and published in 1973. The Committee has determined that the criteria be revised at this time, that the term juvenile rheumatoid arthritis be retained, and that the disease should be classified into three subtypes based on onset (systemic, polyarticular, and pauciarticular). Confusion would result from attempts at this time to change the name to juvenile chronic polyarthritis and to create subclassifications. The term Still's disease, which pays legitimate homage to Dr. George Frederick Still, has nonetheless become confusing in that it denotes different clinical patterns in different parts of the world, and it should not be used. The purpose of a working classification is to allow clinicians everywhere to report experiences that can be readily categorized. A classification with appropriate subtypes allows uniform multicenter evaluation of patients with different manifestations. Clusters can then be identified with regard to prognosis or therapy. The following classification enumerates requirements for a diagnosis of JRA and three subtypes based on clinical onset.
B. General criteria for juvenile rheumatoid arthritis. Persistent arthritis of one or more joints for at least 6 weeks is sufficient for diagnosis if the conditions listed under exclusions have been eliminated. Arthritis is defined as swelling of a joint or limitation of motion with heat, pain, or tenderness. Pain or tenderness alone is not sufficient for the diagnosis of arthritis. Joints are counted individually with certain exceptions. The cervical spine is considered one joint. The carpal joints of each hand are counted as one joint, as are the tarsal joints on each foot. The metacarpophalangeal, metatarsophalangeal, and proximal and distal interphalangeal joints are counted individually.
C. Subtypes of juvenile rheumatoid arthritis according to onset. The onset subtype is determined by manifestations during the first 6 months of disease. Although manifestations more closely resembling those of another onset subtype may appear later, the subtype present during the initial 6 months remains the onset subtype.
1. Systemic-onset juvenile rheumatoid arthritis. This subtype is defined as JRA with persistent intermittent fever (daily intermittent temperature elevations to 103°F or more) with or without rheumatoid rash or other organ involvement. Typical fever and rash are considered probable systemic-onset JRA if not associated with arthritis. Before a definite diagnosis can be made, arthritis as defined must be present.
2. Pauciarticular-onset juvenile rheumatoid arthritis. This subtype is defined as JRA with arthritis in four or fewer joints. Patients with systemic-onset JRA are excluded from this onset subtype.
3. Polyarticular-onset juvenile rheumatoid arthritis. This subtype is defined as JRA with arthritis in five or more joints. Patients with systemic-onset JRA are excluded from this subtype.
4. Other factors to be considered for purposes of continuing study. The following factors may be important for better understanding and future classification:
a. Rheumatoid factors.
b. Antinuclear antibodies (ANAs).
c. Histocompatibility antigens.
f. Iridocyclitis (chronic or acute).
h. Number of affected joints.
i. Distribution of affected joints.
j. Family history of arthritis and related rheumatic manifestations. k. Responses to drug therapy. l. Immunologic abnormalities.
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