J Tiao1,2, R Feng3, E M Berger4, J F Brandsema5, C C Coughlin6, N Khan1,2, E A Kichula5, M A Lerman7, S Lvovich8, P J McMahon9, L G Rider10, A I Rubin1,9, L V Scalzi11, D M Smith5, A J Taxter12, J R Treat9, R P Williams13, S W Yum5, J Okawa1, V P Werth1,2. 1. Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, U.S.A. 2. Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, PA, U.S.A. 3. Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania, Philadelphia, PA, U.S.A. 4. Hackensack University Medical Center, Hackensack, NJ, U.S.A. 5. Division of Neurology, Children's Hospital of Philadelphia, Philadelphia, PA, U.S.A. 6. Division of Dermatology, Washington University School of Medicine, St Louis, MO, U.S.A. 7. Division of Rheumatology, Children's Hospital of Philadelphia, Philadelphia, PA, U.S.A. 8. St Christopher's Hospital for Children, Philadelphia, PA, U.S.A. 9. Division of Dermatology, Children's Hospital of Philadelphia, Philadelphia, PA, U.S.A. 10. Environmental Autoimmunity Group. Clinical Research Branch, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, U.S.A. 11. Penn State Children's Hospital, Hershey, PA, U.S.A. 12. Brenner Children's Hospital, Wake Forest Baptist Medical Center, Winston-Salem, NC, U.S.A. 13. Minneapolis Clinic of Neurology, Maple Grove, MN, U.S.A.
Abstract
BACKGROUND: The Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) and Cutaneous Assessment Tool-Binary Method (CAT-BM) have been shown to be reliable and valid outcome measures to assess cutaneous disease in adult dermatomyositis (DM) and juvenile DM (JDM), respectively. OBJECTIVES: This study compared the CDASI and CAT-BM for use by paediatric dermatologists, paediatric rheumatologists and paediatric neurologists in patients with JDM. METHODS: Five paediatric dermatologists, five paediatric rheumatologists and five paediatric neurologists each evaluated 14 patients with JDM using the CDASI, CAT-BM, and skin Physician Global Assessment (PGA) scales. Inter-rater reliability, intra-rater reliability, construct validity and completion time were compared. RESULTS: Inter-rater reliability for CDASI activity and damage scores was good to moderate for paediatric dermatologists and rheumatologists, but poor for paediatric neurologists. The inter-rater reliability for CAT-BM activity scores was moderate for paediatric dermatologists and rheumatologists, but poor for paediatric neurologists and poor across all specialties for damage scores. Intra-rater reliability for the CDASI and CAT-BM activity and damage scores was moderate to excellent for paediatric dermatologists, rheumatologists and neurologists. Strong associations were found between skin PGA activity and damage scores and CDASI or CAT-BM activity and damage scores, respectively (P < 0·002). The CDASI had a mean completion time of 5·4 min compared with that for the CAT-BM of 3·1 min. CONCLUSIONS: Our data confirm the reliability of the CDASI activity and damage scores and the CAT-BM activity scores when used by paediatric dermatologists and rheumatologists in assessing JDM. Significant variation existed in the paediatric neurologists' scores.
BACKGROUND: The Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) and Cutaneous Assessment Tool-Binary Method (CAT-BM) have been shown to be reliable and valid outcome measures to assess cutaneous disease in adult dermatomyositis (DM) and juvenile DM (JDM), respectively. OBJECTIVES: This study compared the CDASI and CAT-BM for use by paediatric dermatologists, paediatric rheumatologists and paediatric neurologists in patients with JDM. METHODS: Five paediatric dermatologists, five paediatric rheumatologists and five paediatric neurologists each evaluated 14 patients with JDM using the CDASI, CAT-BM, and skin Physician Global Assessment (PGA) scales. Inter-rater reliability, intra-rater reliability, construct validity and completion time were compared. RESULTS: Inter-rater reliability for CDASI activity and damage scores was good to moderate for paediatric dermatologists and rheumatologists, but poor for paediatric neurologists. The inter-rater reliability for CAT-BM activity scores was moderate for paediatric dermatologists and rheumatologists, but poor for paediatric neurologists and poor across all specialties for damage scores. Intra-rater reliability for the CDASI and CAT-BM activity and damage scores was moderate to excellent for paediatric dermatologists, rheumatologists and neurologists. Strong associations were found between skin PGA activity and damage scores and CDASI or CAT-BM activity and damage scores, respectively (P < 0·002). The CDASI had a mean completion time of 5·4 min compared with that for the CAT-BM of 3·1 min. CONCLUSIONS: Our data confirm the reliability of the CDASI activity and damage scores and the CAT-BM activity scores when used by paediatric dermatologists and rheumatologists in assessing JDM. Significant variation existed in the paediatric neurologists' scores.
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