Evaluation of the Reliability of the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) and Cutaneous Assessment Tool Binary Method (CAT-BM) in Juvenile Dermatomyositis Among Pediatric Dermatologists, Rheumatologists, and Neurologists

Janice Tiao^1,2, Rui Feng³, Emily M. Berger⁴, John F. Brandsema⁵, Carrie C. Coughlin⁶, Neelam Khan², Elizabeth A. Kichula⁵, Melissa A. Lerman⁷, Svetlana Lvovich⁸, Patrick J. McMahon⁹, Lisa G. Rider¹⁰, Adam I. Rubin², Lisabeth V. Scalzi¹¹, Douglas M. Smith⁵, Alysha J. Taxter¹², James R. Treat⁹, Ryan P. Williams¹³, Sabrina W. Yum⁵, Joyce Okawa² and Victoria P. Werth^1,2, ¹Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, PA, ²Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, ³Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania, Philadelphia, PA, ⁴Hackensack University Medical Center, Hackensack, NJ, ⁵Division of Neurology, Children's Hospital of Philadelphia, Philadelphia, PA, ⁶Division of Dermatology, Washington University School of Medicine, St. Louis, MO, ⁷Division of Rheumatology, Children's Hospital of Philadelphia, Philadelphia, PA, ⁸St. Christopher's Hospital for Children, Philadelphia, PA, ⁹Division of Dermatology, Children's Hospital of Philadelphia, Philadelphia, PA, ¹⁰Environmental Autoimmunity Group, Program of Clinical Research, National Institute of Environmental Health Sciences, National Institutes of Health, US Department of Health and Human Services, Bethesda, MD, ¹¹Department of Rheumatology, Penn State Hershey Children’s Hospital, Hershey, PA, ¹²Pediatrics, Brenner Children's Hospital, Wake Forest Baptist Medical Center, Winston-Salem, NC, ¹³Minneapolis Clinic of Neurology, Maple Grove, MN

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: juvenile dermatomyositis and outcome measures

Session Information

Date: Monday, November 14, 2016

Title: Pediatric Rheumatology – Clinical and Therapeutic Aspects - Poster II: Myositis, Systemic Lupus Erythematosus, Sjögren's Syndrome

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: 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. This study compared the CDASI and CAT-BM for use by pediatric dermatologists, pediatric rheumatologists, and pediatric neurologists in patients with JDM.

Methods: Five pediatric dermatologists, five pediatric rheumatologists, and five pediatric neurologists each evaluated 14 patients with JDM using the CDASI, CAT-BM, and skin Physician Global Assessment (PGA) scales. Inter-rater, 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 pediatric dermatologists and rheumatologists, but poor for pediatric neurologists. The inter-rater reliability for CAT-BM activity scores was moderate for pediatric dermatologists and rheumatologists, but poor for pediatric 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 pediatric 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 5.1 minutes versus the CAT-BM of 2.8 minutes.

Conclusion: Our data confirm the reliability of the CDASI activity and damage scores and the CAT-BM activity scores when used by pediatric dermatologists and rheumatologists in assessing JDM. Significant variation existed in the pediatric neurologistsÕ scores.

Table 1. Inter-rater reliability of Skin Assessment Tool Scores in JDM patients among pediatric specialists*

	Within pediatric dermatologists (n=5)	Within pediatric rheumatologists (n=5)	Within pediatric neurologists (n=5)

CDASI
Activity	0.52 (0.44-0.61)	0.81 (0.76-0.85)	0.47 (0.39-0.56)
Damage	0.59 (0.51-0.67)	0.59 (0.51-0.66)	0.46 (0.37-0.54)
CAT-BM
Activity	0.58 (0.50-0.66)	0.67 (0.60-0.74)	0.42 (0.33-0.51)
Damage	0.49 (0.41-0.58)	0.48 (0.39-0.57)	0.29 (0.20-0.38)
Skin PGA
Activity	0.64 (0.50-0.78)	0.47 (0.39-0.56)	0.38 (0.29-0.47)
Damage	0.71 (0.65-0.83)	0.31 (0.22-0.40)	0.73 (0.67-0.79)

*Intra-class correlation coefficients (95% C.I.) Abbreviations: Abbreviations: CDASI: Cutaneous Dermatomyositis Disease Area and Severity Index; CAT-BM: Cutaneous Assessment Tool-Binary Method; PGA: Physician Global Assessment

Table 2. Intra-rater reliability of Skin Assessment Tool Scores in JDM patients among pediatric specialists*

	Within pediatric dermatologists (n=5)	Within pediatric rheumatologists (n=5)	Within pediatric neurologists (n=5)

CDASI
Activity	0.90 (0.78-1.00)	0.91 (0.79-1.00)	0.65 (0.29-1.00)
Damage	0.97 (0.94-1.00)	0.64 (0.27-1.00)	0.78 (0.53-1.00)
CAT-BM
Activity	0.86 (0.70-1.00)	0.88 (0.74-1.00)	0.91 (0.80-1.00)
Damage	0.93 (0.85-1.00)	0.73 (0.44-1.00)	0.89 (0.76-1.00)
Skin PGA
Activity	0.95 (0.90-1.00)	0.50 (0.03-0.96)	0.83 (0.64-1.00)
Damage	0.84 (0.66-1.00)	0.55 (0.12-0.98)	0.96 (0.91-1.00)

Disclosure: J. Tiao, None; R. Feng, None; E. M. Berger, None; J. F. Brandsema, None; C. C. Coughlin, None; N. Khan, None; E. A. Kichula, None; M. A. Lerman, None; S. Lvovich, None; P. J. McMahon, None; L. G. Rider, None; A. I. Rubin, None; L. V. Scalzi, None; D. M. Smith, None; A. J. Taxter, None; J. R. Treat, None; R. P. Williams, None; S. W. Yum, None; J. Okawa, None; V. P. Werth, None.

To cite this abstract in AMA style:

Tiao J, Feng R, Berger EM, Brandsema JF, Coughlin CC, Khan N, Kichula EA, Lerman MA, Lvovich S, McMahon PJ, Rider LG, Rubin AI, Scalzi LV, Smith DM, Taxter AJ, Treat JR, Williams RP, Yum SW, Okawa J, Werth VP. Evaluation of the Reliability of the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) and Cutaneous Assessment Tool Binary Method (CAT-BM) in Juvenile Dermatomyositis Among Pediatric Dermatologists, Rheumatologists, and Neurologists [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/evaluation-of-the-reliability-of-the-cutaneous-dermatomyositis-disease-area-and-severity-index-cdasi-and-cutaneous-assessment-tool-binary-method-cat-bm-in-juvenile-dermatomyositis-among-pediatric/. Accessed .

« Back to 2016 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/evaluation-of-the-reliability-of-the-cutaneous-dermatomyositis-disease-area-and-severity-index-cdasi-and-cutaneous-assessment-tool-binary-method-cat-bm-in-juvenile-dermatomyositis-among-pediatric/