Criteria for the classification of juvenile rheumatoid arthritis were analyzed in a detailed database of 250 children in order to assess the accuracy of diagnosis and validity of onset types and course subtypes. A number of conclusions have been derived from this study: 1) All definitions of the 1973 criteria for classification of juvenile rheumatoid arthritis should be retained. 2) The addition of onset types to the 1976 revision of the criteria has been validated. 3) The course of the disease after the onset period of 6 months is as important to the outcome of a group of children as is the onset type. 4) The current classification should be broadened to include the course subtypes.
The CMAS demonstrated an acceptable range of observed scores, excellent convergent validity, and excellent inter- and intrarater reliability. The CMAS is validated to quantitatively assess muscle function in the areas of strength and endurance in children with idiopathic inflammatory myopathies. It can be used in routine clinical care as well as therapeutic trials.
Childhood dermatomyositis is a distinct subset of dermatomyositis with highly variable outcome. We reviewed our experience with 29 patients observed over a period of 22 years and attempted to correlate tissue manifestations with outcome. Distinctive vascular lesions included non-necrotizing lymphocytic vasculitis and a unique spectrum of endovascular injury producing temporary or permanent occlusion of small arteries and capillaries. Vessels with nonineammatory endovasculopathy were often reactive with fluorescein-conjugated antisera to IgM, C3d, and/or fibrin. Lesions linked to endovascular injury include infarction, zonal myopathy , and loss of capillary network. We were able to identify half of the children destined to have persistent morbidity on the basis of severity of vasculopathy in pretreatment muscle-biopsy specimens. Our observations support a central role for endothelial cell injury in the pathogenesis of childhood dermatomyositis, suggest a basis for
Two questionnaires were developed for measuring disability due to juvenile rheumatoid arthritis (JRA), one based on patient reports and one on parent reports. These questionnaires were termed the Juvenile Arthritis Functional Assessment Report for Children (JAFAR-C) and for Parents (JAFAR-P). The questionnaires were administered to 72 JRA patients ages 7-18 years and to their parents. Respondents rated the patient's recent ability to perform 23 activities. Patient reports and parent reports were found to correlate highly with each other and with an objective assessment performed by therapists. Questionnaire scores did not correlate significantly with the age of the patient. The JAFAR appears to be a convenient, reliable, and valid measure of disability in patients with JRA.
A disability assessment tool, the Juvenile Arthritis Functional Assessment Scale, was developed for, and validated in, patients with juvenile rheumatoid arthritis (JRA). Standards for this 10-item tool were developed using the scores of 63 normal school children as controls and comparing these results with those of 71 agematched JRA patients (age 7-16 years). The JRA patients scored statistically significantly higher on the scale, which also demonstrated excellent internal and convergent validity and internal reliability. The test is easily administered in 10 minutes by a physical or occupational therapist in a clinical or office setting. This tool represents the first normalized disability assessment tool developed for JRA patients.Juvenile rheumatoid arthritis (JRA) is a complex disease of varying severity and prognosis. It is the most frequently encountered pediatric rheumatic disease, one of the more common chronic diseases of
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