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The Cutaneous Assessment Tool: development and reliability in juvenile idiopathic inflammatory myopathy
IWK Health Centre and Dalhousie University, Halifax, Nova Scotia, Canada, 1Washington Hospital Center, Washington, DC, 2Center for Biologics Evaluation and Research, Food and Drug Administration, DHHS, Rockville, MD, USA, 3Hospital For Sick Children and University of Toronto, Toronto, Ontario, Canada, 4Texas Children's Hospital and Baylor College of Medicine, Houston, TX, 5Connecticut Children's Medical Center and University of Connecticut, Hartford, CT, 6University of Kansas, Kansas City, KS, 7Columbus Children's Hospital and Ohio State University, Columbus, OH, 8Children's Hospital and University of Washington, Seattle, WA, 9Children's Hospital and University of Cincinnati, Cincinnati, OH, USA, 10Mayo Clinic, Rochester, 11Riley Children's Hospital and Indiana University School of Medicine, Indianapolis and 12Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, North Carolina.
Correspondence to: A. M. Huber, MD, Division of Pediatric Rheumatology, IWK Health Centre, 5850 University Avenue, Halifax, Nova Scotia, Canada B3K 6R8 E-mail: adam.huber{at}iwk.nshealth.ca
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Abstract |
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Objectives. Clinical care and therapeutic trials in idiopathic inflammatory myopathies (IIM) require accurate and consistent assessment of cutaneous involvement. The Cutaneous Assessment Tool (CAT) was designed to measure skin activity and damage in IIM. We describe the development and inter-rater reliability of the CAT, and the frequency of lesions endorsed in a large population of juvenile IIM patients.
Methods. The CAT includes 10 activity, 4 damage and 7 combined lesions. Thirty-two photographic slides depicting IIM skin lesions were assessed by 11 raters. One hundred and twenty-three children were assessed by 11 paediatric rheumatologists at 10 centres. Inter-rater reliability was assessed using simple agreements and intra-class correlation coefficients (ICC).
Results. Simple agreements in recognizing lesions as present or absent were generally high (0.5–1.0). ICCs for CAT lesions were moderate (0.4–0.75) in both slides and real patients. ICCs for the CAT activity and damage scores were 0.71 and 0.81, respectively. CAT activity scores ranged from 0 to 44 (median 7, potential range 0–96) and CAT damage scores ranged from 0 to 13 (median 1, potential range 0–22). The most common cutaneous lesions endorsed were periungual capillary loop changes (63%), Gottron's papules/sign (53%), heliotrope rash (49%) and malar/facial erythema (49%).
Conclusions. Total CAT activity and damage scores have moderate to good reliability. Assessors generally agree on the presence of a variety of cutaneous lesions. The CAT is a promising, semi-quantitative tool to comprehensively assess skin disease activity and damage in IIM.
KEY WORDS: Juvenile idiopathic inflammatory myopathy, Juvenile dermatomyositis, Skin disease, Cutaneous Assessment Tool, Assessment
*Contributors to this study are listed in the appendix.
Present address: P. A. Lachenbruch, Department of Public Health, Oregon State University, Corvallis, OR, USA.
Submitted 5 December 2006;
revised version accepted 25 April 2007.
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