Introduction Although many children with inflammatory arthritis enter long-term remission before entering adulthood, a substantial proportion exhibit persistent disease and require ongoing rheumatologic care beyond the pediatric years. These patients have medical needs that differ, occasionally substantially, from those of patients with adult-onset rheumatic diseases. This review will summarize outcomes data in juvenile-onset arthritis and discuss the medical management of these patients as adults, including the transition from pediatric to adult care.

OBJECTIVE To document and evaluate the scores that normal, healthy children achieve when performing 9 maneuvers of the Childhood Myositis Assessment Scale (CMAS). METHODS A total of 303 healthy children, 4-9 years of age, were scored as they performed 9 CMAS maneuvers. The data were then evaluated to determine whether normal scores for some maneuvers are age and sex dependent. RESULTS All children were able to achieve maximum possible scores for the supine to prone, supine to sit, floor sit, floor rise, and chair rise maneuvers. All but 2 4-year-olds achieved a maximum possible score for the arm raise/duration maneuver. Performance of the head lift and sit-up maneuvers varied significantly, depending primarily on age. Children in all age groups had less difficulty performing the leg lift than the head lift or sit-up. CONCLUSION The normative data generated by this study are of value for interpreting the serial CMAS scores of children with idiopathic inflammatory myopathies.

OBJECTIVETo examine the measurement characteristics of the Childhood Myositis Assessment Scale (CMAS) in children with juvenile idiopathic inflammatory myopathy (juvenile IIM), and to obtain preliminary data on the clinical significance of CMAS scores.METHODSOne hundred eight children with juvenile IIM were evaluated on 2 occasions, 7-9 months apart, using various measures of physical function, strength, and disease activity. Interrater reliability, construct validity, and responsiveness of the CMAS were examined. The minimum clinically important difference (MID) and CMAS scores corresponding to various degrees of physical disability were estimated.RESULTSThe intraclass correlation coefficient for 26 patients assessed by 2 examiners was 0.89, indicating very good interrater reliability. The CMAS score correlated highly with the Childhood Health Assessment Questionnaire (C-HAQ) score and with findings on manual muscle testing (MMT) (r(s) = -0.73 and 0.73, respectively) and moderately with physician-assessed global disease activity and skin activity, parent-assessed global disease severity, and muscle magnetic resonance imaging (r(s) = -0.44 to -0.61), thereby demonstrating good construct validity. The standardized response mean was 0.81 (95% confidence interval 0.53, 1.09) in patients with at least 0.8 cm improvement on a 10-cm visual analog scale for physician-assessed global disease activity, indicating strong responsiveness. In bivariate regression models predicting physician-assessed global disease activity, MMT remained significant in models containing the CMAS (P = 0.03) while the C-HAQ did not (P = 0.4). Estimates of the MID ranged from 1.5 to 3.0 points on a 0-52-point scale. CMAS scores corresponding to no, mild, mild-to-moderate, and moderate physical disability, respectively, were 48, 45, 39, and 30.CONCLUSIONThe CMAS exhibits good reliability, construct validity, and responsiveness, and is therefore a valid instrument for the assessment of physical function, muscle strength, and endurance in children with juvenile IIM. Preliminary data on MID and corresponding levels of disability should aid in the clinical interpretation of CMAS scores when assessing patients with juvenile IIM.

OBJECTIVE:To examine the validity of the Childhood Health Assessment Questionnaire (CHAQ) in patients with juvenile idiopathic inflammatory myopathy (IIM). METHODS:One hundred fifteen patients were enrolled in a multicenter collaborative study, during which subjects were assessed twice, 7-9 months apart. Physical function was measured using the CHAQ. Internal reliability was assessed using adjusted item-total correlations and item endorsement rates. Construct validity was assessed by comparing predicted and actual correlations of the CHAQ with other measures of physical function and disease activity. Responsiveness was assessed by calculating effect size (ES) and standardized response mean (SRM) in a group of a priori defined "improvers." RESULTS:Item-total correlations were high (rs range = 0.35-0.81), suggesting all items were related to overall physical function. Manual muscle testing and the Childhood Myositis Assessment Scale correlated moderate to strongly with the CHAQ (r = -0.64 and -0.75, both p < 0.001). Moderate correlations were also seen with the physician global assessment of disease activity (rs = 0.58, p < 0.001), parent global assessment of overall health (rs = -0.65, p < 0.001), Steinbrocker function class (rs = 0.69, p < 0.001), and global skin activity (rs = 0.40, p < 0.001), while global disease damage and skin damage had low correlations (rs = 0.13 and 0.07, p > or =0.17). Responsiveness of the CHAQ was high, with ES = 1.05 and SRM = 1.20. CONCLUSION:In this large cohort of patients with juvenile IIM, the CHAQ exhibited internal reliability, construct validity, and strong responsiveness. We conclude that the CHAQ is a valid measure of physical function in juvenile IIM, appropriate for use in therapeutic trials, and potentially in the clinical care of these patients.

PURPOSE:To study the effect of exercise on short inversion time inversion-recovery (STIR) magnetic resonance (MR) images of thigh muscles in children with juvenile idiopathic inflammatory myopathy. MATERIALS AND METHODS:Thirty-two MR studies were performed in 19 patients with juvenile idiopathic inflammatory myopathy who performed stair-stepping exercise for up to 10 minutes (mean, 5.7 minutes). Baseline T1-weighted (n = 32) and STIR (n = 32) images and STIR images immediately (n = 32) and at 30 (n = 24) and 60 (n = 29) minutes after exercise were obtained at 0.5 T. Four radiologists graded STIR signal intensity changes, in observer performance experiments in which they were blinded to the order of image acquisition in relation to exercise. RESULTS:Changes in muscle signal intensity were observed on STIR images obtained immediately after exercise in 20 of 32 (63%) studies. The mean signal intensity score immediately after exercise (1.7 +/- 1.0 [SD]) increased compared with the mean baseline score (1.4 +/- 1.1) (P = .0005) and resolved by 30 minutes after exercise. The magnitude of exercise-induced changes correlated with the amount of work performed (r = 0.51, P = .003) but not with disease activity or baseline signal intensity when the changes were corrected for work (r < 0.17, P > .35). Radiologists demonstrated moderate to substantial agreement in the grading of signal intensity changes after exercise (kappa = 0.60-0.84). CONCLUSION:In patients with juvenile idiopathic inflammatory myopathy, stair-stepping exercise induces signal intensity changes on STIR MR studies of muscle for approximately 30 minutes after exercise, in a distribution that may mimic active muscle inflammation.

Journal of School HealthVolume 57, Issue 4 p. 162-164 A Needs Assessment of Children with Rheumatic Diseases Elizabeth Stoff, Elizabeth Stoff Elizabeth Stoff, MEd, Education Coordinator, Children's Hospital National Medical Center, 111 Michigan Ave., NW, Washington, DC 20010Search for more papers by this authorSherry D. Molock, Sherry D. Molock Patience H. White, MD, Assistant Professor, Child Health and Development and Medicine, and Director, Comprehensive Pediatric Rheumatology Center, Children's Hospital National Medical Center, 111 Michigan Ave., NW, Washington, DC 20010Search for more papers by this authorPatience H. White, Patience H. White Sherry D. Molock, PhD, Assistant Professor, Dept. of Psychology, School of Sciences, Auburn University of Montgomery, Montgomery, AL 36193-0401.Search for more papers by this author Elizabeth Stoff, Elizabeth Stoff Elizabeth Stoff, MEd, Education Coordinator, Children's Hospital National Medical Center, 111 Michigan Ave., NW, Washington, DC 20010Search for more papers by this authorSherry D. Molock, Sherry D. Molock Patience H. White, MD, Assistant Professor, Child Health and Development and Medicine, and Director, Comprehensive Pediatric Rheumatology Center, Children's Hospital National Medical Center, 111 Michigan Ave., NW, Washington, DC 20010Search for more papers by this authorPatience H. White, Patience H. White Sherry D. Molock, PhD, Assistant Professor, Dept. of Psychology, School of Sciences, Auburn University of Montgomery, Montgomery, AL 36193-0401.Search for more papers by this author First published: April 1987 https://doi.org/10.1111/j.1746-1561.1987.tb04166.xCitations: 4 AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat REFERENCES 1 Arthreya B., Ingall C.: Juvenile Rheumatoid Arthritis, in J. Fifthian (ed): Understanding the Child with a Chronic Illness in the Classroom. Phoenix, Oryx Press, 1984, pp 93–104. 2 Brewer E., Giannini E., Person D.: Juvenile Rheumatoid Arthritis. Philadelphia, Saunders Press, 1970, p 2. 3 Travis G.: Chronic Illness in Children. Stanford, CA, Stanford University Press, 1976, p 5. 4 US Dept of Education: Digest of Educational Statistics. National Center for Educational Statistics, US Dept of Education, 19831984. 5 Walker D., Jacobs F.: Public School Programs for Chronically ill children, in N. Hobbs, J. Perrin (eds.): Issues in the Care of Children with Chronic Illness. San Francisco, Jossey-Bass Publishers, 1985, pp 615–655. 6 Baird S., Ashcroft S.: Need-based Educational Policy for Chroncially Ill Children, in N. Hobbs, J. Perrin (eds.): Issues in the Care of Children with Chronic Illness. San Francisco, Jossey-Bass Publishers, 1985, pp 656–671. 7 Vitello S.: The Tatro case: Who gets what and why. Except Child 1986; 52(4): 353–356. Citing Literature Volume57, Issue4April 1987Pages 162-164 ReferencesRelatedInformation