Currently recommended skin scores correlate highly in the assessment of patients with Juvenile Dermatomyositis (JDM)

Abstract Background Juvenile Dermatomyositis (JDM) is a rare, chronic, and life-threatening childhood autoimmune disease. Currently, there are recommended, reliable and validated measurement tools for assessment of skin disease activity in JDM including the Disease Activity Score (skinDAS), Cutaneou...

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Main Authors: Alexander Gebreamlak, Katherine M. Sawicka, Rose Garrett, Y. Ingrid Goh, Kayla M. Baker, Brian M. Feldman
Format: Article
Language:English
Published: BMC 2023-06-01
Series:Pediatric Rheumatology Online Journal
Subjects:
Online Access:https://doi.org/10.1186/s12969-023-00844-5
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author Alexander Gebreamlak
Katherine M. Sawicka
Rose Garrett
Y. Ingrid Goh
Kayla M. Baker
Brian M. Feldman
author_facet Alexander Gebreamlak
Katherine M. Sawicka
Rose Garrett
Y. Ingrid Goh
Kayla M. Baker
Brian M. Feldman
author_sort Alexander Gebreamlak
collection DOAJ
description Abstract Background Juvenile Dermatomyositis (JDM) is a rare, chronic, and life-threatening childhood autoimmune disease. Currently, there are recommended, reliable and validated measurement tools for assessment of skin disease activity in JDM including the Disease Activity Score (skinDAS), Cutaneous Assessment Tool (CAT), and the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI). The Physician’s global assessment skin visual analog scale (Skin VAS) is also widely used for skin activity in JDM. For the purpose of comparative international studies, we wanted to compare these tools to the Physician’s skin VAS (as a standard) to identify which performs better. Objectives We sought to compare the correlations of these scoring tools, and separately assess the responsiveness each tool demonstrates following patient treatment, in order to see if one tool may be preferred. This was determined by assessing how well these tools correlate with each other, and the Physician’s skin VAS over time, as well as the responsiveness of each tool after patient treatment. Methods Skin scores were recorded at a baseline (first visit after June 1st, 2018) and all follow-up office visits at the Juvenile Dermatomyositis Clinic. Following baseline visits, patients were followed up as clinically indicated. A subset of newly diagnosed patients (inception cohort) was identified. Correlations were assessed at the baseline visit and over time for the whole cohort. The correlations over time were derived using Generalized Estimating Equations (GEEs). Standardized response means with 95% confidence intervals were calculated to test score responsiveness for the nested inception cohort. Results The skinDAS, CAT and CDASI all correlated highly with each other and with the Physician’s skin VAS. The three scoring tools accurately reflected Physician’s skin VAS scores over time. In addition, all tools showed moderate to high responsiveness following treatment. Conclusion All studied skin score tools performed well in our study and appear to be useful. Since no tool far outperforms the others, arbitrary consensus will be needed to select a single standard measurement tool for the purposes of efficiency and global comparability.
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spelling doaj.art-9567a2ce053d43328753d39195d8cf562023-07-02T11:10:10ZengBMCPediatric Rheumatology Online Journal1546-00962023-06-012111810.1186/s12969-023-00844-5Currently recommended skin scores correlate highly in the assessment of patients with Juvenile Dermatomyositis (JDM)Alexander Gebreamlak0Katherine M. Sawicka1Rose Garrett2Y. Ingrid Goh3Kayla M. Baker4Brian M. Feldman5Child Health Evaluative Sciences Program, SickKids Research InstituteChild Health Evaluative Sciences Program, SickKids Research InstituteChild Health Evaluative Sciences Program, SickKids Research InstituteChild Health Evaluative Sciences Program, SickKids Research InstituteChild Health Evaluative Sciences Program, SickKids Research InstituteChild Health Evaluative Sciences Program, SickKids Research InstituteAbstract Background Juvenile Dermatomyositis (JDM) is a rare, chronic, and life-threatening childhood autoimmune disease. Currently, there are recommended, reliable and validated measurement tools for assessment of skin disease activity in JDM including the Disease Activity Score (skinDAS), Cutaneous Assessment Tool (CAT), and the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI). The Physician’s global assessment skin visual analog scale (Skin VAS) is also widely used for skin activity in JDM. For the purpose of comparative international studies, we wanted to compare these tools to the Physician’s skin VAS (as a standard) to identify which performs better. Objectives We sought to compare the correlations of these scoring tools, and separately assess the responsiveness each tool demonstrates following patient treatment, in order to see if one tool may be preferred. This was determined by assessing how well these tools correlate with each other, and the Physician’s skin VAS over time, as well as the responsiveness of each tool after patient treatment. Methods Skin scores were recorded at a baseline (first visit after June 1st, 2018) and all follow-up office visits at the Juvenile Dermatomyositis Clinic. Following baseline visits, patients were followed up as clinically indicated. A subset of newly diagnosed patients (inception cohort) was identified. Correlations were assessed at the baseline visit and over time for the whole cohort. The correlations over time were derived using Generalized Estimating Equations (GEEs). Standardized response means with 95% confidence intervals were calculated to test score responsiveness for the nested inception cohort. Results The skinDAS, CAT and CDASI all correlated highly with each other and with the Physician’s skin VAS. The three scoring tools accurately reflected Physician’s skin VAS scores over time. In addition, all tools showed moderate to high responsiveness following treatment. Conclusion All studied skin score tools performed well in our study and appear to be useful. Since no tool far outperforms the others, arbitrary consensus will be needed to select a single standard measurement tool for the purposes of efficiency and global comparability.https://doi.org/10.1186/s12969-023-00844-5Juvenile dermatomyositis (JDM)Disease activity score skin subscale (skinDAS)Cutaneous assessment tool (CAT)Cutaneous dermatomyositis disease area and severity index (CDASI)Physician’s global assessment skin visual analog scale (Skin VAS)Patient outcomes
spellingShingle Alexander Gebreamlak
Katherine M. Sawicka
Rose Garrett
Y. Ingrid Goh
Kayla M. Baker
Brian M. Feldman
Currently recommended skin scores correlate highly in the assessment of patients with Juvenile Dermatomyositis (JDM)
Pediatric Rheumatology Online Journal
Juvenile dermatomyositis (JDM)
Disease activity score skin subscale (skinDAS)
Cutaneous assessment tool (CAT)
Cutaneous dermatomyositis disease area and severity index (CDASI)
Physician’s global assessment skin visual analog scale (Skin VAS)
Patient outcomes
title Currently recommended skin scores correlate highly in the assessment of patients with Juvenile Dermatomyositis (JDM)
title_full Currently recommended skin scores correlate highly in the assessment of patients with Juvenile Dermatomyositis (JDM)
title_fullStr Currently recommended skin scores correlate highly in the assessment of patients with Juvenile Dermatomyositis (JDM)
title_full_unstemmed Currently recommended skin scores correlate highly in the assessment of patients with Juvenile Dermatomyositis (JDM)
title_short Currently recommended skin scores correlate highly in the assessment of patients with Juvenile Dermatomyositis (JDM)
title_sort currently recommended skin scores correlate highly in the assessment of patients with juvenile dermatomyositis jdm
topic Juvenile dermatomyositis (JDM)
Disease activity score skin subscale (skinDAS)
Cutaneous assessment tool (CAT)
Cutaneous dermatomyositis disease area and severity index (CDASI)
Physician’s global assessment skin visual analog scale (Skin VAS)
Patient outcomes
url https://doi.org/10.1186/s12969-023-00844-5
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