Evaluation of the reliability of the Cutaneous Dermatomyositis Disease Area and Severity Index and the Cutaneous Assessment Tool-Binary Method in juvenile dermatomyositis among paediatric dermatologists, rheumatologists and neurologists

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. Objective 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 5.4 minutes versus the CAT-BM of 3.1 minutes. Conclusions 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 neurologists’ scores.

Evaluation of the reliability and validity of the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) in paediatric cutaneous lupus among paediatric dermatologists and rheumatologists

“…Our study had several limitations, including the small sample size of 11 patients. However, despite the small sample size, the inter‐ and intrarater reliabilities for activity were still excellent, and the results are similar to those achieved in other validation studies . Another limitation is that recall bias may have been introduced during the rerater session, affecting intrarater reliability.…”

“…The skin PGA is a tool that estimates overall skin disease severity and has been used in multiple validation studies for skin diseases . It requires physicians to score the patient's skin‐related health on a 0–10 scale (0 = worst skin condition imaginable, 10 = perfect health) for his or her overall skin score, skin activity and skin damage.…”

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“…Another prior research study similar to the current research study conducted in approach and methodology have revealed and displayed that CLASI activity scoring system had excellent inter-rater reliability in comparison to PGA, that revealed good inter-rater reliability. Interestingly it is well known that, the activity scoring is a www.opastonline.com more a more valuable measure Than damage scoring since current skin disease activity levels is measured in clinical trials [2,4,9]. Furthermore, in harmony and great similarity to the current research study it was shown that generally CLASI scoring system is more trustworthy than tool than the PGA system especially in evaluation of children diseases activity levels [11,13].…”

“…Cutaneous lupus in pediatric patients is a chief issue causing morbidity, quality of life impairment and psychosocial insults. Proper assessment permits proper and improved management protocols evaluation therefore aiding in enhancement level of care in those categories of cases among pediatric population [9,10].…”

Development of a consensus core dataset in juvenile dermatomyositis for clinical use to inform research

“…Recent published work evaluating the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) and the Cutaneous Assessment Tool Binary Method (CAT-BM) in JDM confirms the reliability of both tools when used by paediatric dermatologists or rheumatologists. 49 …”