Development and validation of a composite disease activity score for measurement of muscle and skin involvement in juvenile dermatomyositis

the Pediatric Rheumatology International Trials Organization (PRINTO)

Research output: Contribution to journalArticle

Abstract

Objective. To develop a composite DAS for JDM and provide preliminary evidence of its validity. Methods. The Juvenile DermatoMyositis Activity Index (JDMAI) is composed of four items: physician's global assessment of overall disease activity; parent's/child's global assessment of child's wellbeing; measurement of muscle strength; and assessment of skin disease activity. The score of the JDMAI is the arithmetic sum of the scores of each individual component. Six versions of the JDMAI were tested, which differed in the tools used to assess the third and fourth items. Validation procedures were conducted using three large multinational patient samples including a total of 627 patients. Results. The JDMAI was found to possess face and content validity, good construct validity, satisfactory internal consistency (Cronbach's alpha = 0.58-0.89), fair responsiveness to clinically important change (standardized response mean = 0.82-3.12 among patients improved) and strong capacity to discriminate patients judged as being in the state of inactive disease or low, moderate or high disease activity by the physician (P<0.001) or whose parents were satisfied or not satisfied with the course of their child's illness (P<0.001). Overall, the six versions of the JDMAI showed similar metrological performances in validation analyses. Conclusion. The JDMAI was found to possess good measurement properties in a large population of patients with a wide range of disease activity, and is, therefore, suitable for use in both clinical and research settings. The final version of the JDMAI will be selected after its prospective validation.

Original languageEnglish
Pages (from-to)1196-1205
Number of pages10
JournalRheumatology (United Kingdom)
Volume58
Issue number7
DOIs
Publication statusPublished - Jul 1 2019

Fingerprint

Muscles
Skin
amsonic acid
Parents
Physicians
Muscle Strength
Juvenile dermatomyositis
Skin Diseases
Reproducibility of Results
Research
Population

Keywords

  • Composite disease activity scores
  • Disease activity assessment
  • Idiopathic inflammatory myositis
  • JDM
  • Muscle strength assessment
  • Outcome measures
  • Paediatric rheumatology

ASJC Scopus subject areas

  • Rheumatology
  • Pharmacology (medical)

Cite this

Development and validation of a composite disease activity score for measurement of muscle and skin involvement in juvenile dermatomyositis. / the Pediatric Rheumatology International Trials Organization (PRINTO).

In: Rheumatology (United Kingdom), Vol. 58, No. 7, 01.07.2019, p. 1196-1205.

Research output: Contribution to journalArticle

the Pediatric Rheumatology International Trials Organization (PRINTO). / Development and validation of a composite disease activity score for measurement of muscle and skin involvement in juvenile dermatomyositis. In: Rheumatology (United Kingdom). 2019 ; Vol. 58, No. 7. pp. 1196-1205.
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abstract = "Objective. To develop a composite DAS for JDM and provide preliminary evidence of its validity. Methods. The Juvenile DermatoMyositis Activity Index (JDMAI) is composed of four items: physician's global assessment of overall disease activity; parent's/child's global assessment of child's wellbeing; measurement of muscle strength; and assessment of skin disease activity. The score of the JDMAI is the arithmetic sum of the scores of each individual component. Six versions of the JDMAI were tested, which differed in the tools used to assess the third and fourth items. Validation procedures were conducted using three large multinational patient samples including a total of 627 patients. Results. The JDMAI was found to possess face and content validity, good construct validity, satisfactory internal consistency (Cronbach's alpha = 0.58-0.89), fair responsiveness to clinically important change (standardized response mean = 0.82-3.12 among patients improved) and strong capacity to discriminate patients judged as being in the state of inactive disease or low, moderate or high disease activity by the physician (P<0.001) or whose parents were satisfied or not satisfied with the course of their child's illness (P<0.001). Overall, the six versions of the JDMAI showed similar metrological performances in validation analyses. Conclusion. The JDMAI was found to possess good measurement properties in a large population of patients with a wide range of disease activity, and is, therefore, suitable for use in both clinical and research settings. The final version of the JDMAI will be selected after its prospective validation.",
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T1 - Development and validation of a composite disease activity score for measurement of muscle and skin involvement in juvenile dermatomyositis

AU - the Pediatric Rheumatology International Trials Organization (PRINTO)

AU - Rosina, Silvia

AU - Consolaro, Alessandro

AU - Van Dijkhuizen, Pieter

AU - Pistorio, Angela

AU - Varnier, Giulia Camilla

AU - Bovis, Francesca

AU - Nistala, Kiran

AU - Maillard, Susan

AU - Civino, Adele

AU - Tsitsami, Elena

AU - De Inocencio, Jaime

AU - Jelusic, Marija

AU - Vojinovic, Jelena

AU - Espada, Graciela

AU - Makay, Balahan

AU - Katsicas, Maria Martha

AU - Pratsidou-Gertsi, Polixeni

AU - Lazarevic, Dragana

AU - Rao, Anand Prahalad

AU - Pires Marafon, Denise

AU - Martini, Alberto

AU - Pilkington, Clarissa

AU - Ruperto, Nicolino

AU - Ravelli, Angelo

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N2 - Objective. To develop a composite DAS for JDM and provide preliminary evidence of its validity. Methods. The Juvenile DermatoMyositis Activity Index (JDMAI) is composed of four items: physician's global assessment of overall disease activity; parent's/child's global assessment of child's wellbeing; measurement of muscle strength; and assessment of skin disease activity. The score of the JDMAI is the arithmetic sum of the scores of each individual component. Six versions of the JDMAI were tested, which differed in the tools used to assess the third and fourth items. Validation procedures were conducted using three large multinational patient samples including a total of 627 patients. Results. The JDMAI was found to possess face and content validity, good construct validity, satisfactory internal consistency (Cronbach's alpha = 0.58-0.89), fair responsiveness to clinically important change (standardized response mean = 0.82-3.12 among patients improved) and strong capacity to discriminate patients judged as being in the state of inactive disease or low, moderate or high disease activity by the physician (P<0.001) or whose parents were satisfied or not satisfied with the course of their child's illness (P<0.001). Overall, the six versions of the JDMAI showed similar metrological performances in validation analyses. Conclusion. The JDMAI was found to possess good measurement properties in a large population of patients with a wide range of disease activity, and is, therefore, suitable for use in both clinical and research settings. The final version of the JDMAI will be selected after its prospective validation.

AB - Objective. To develop a composite DAS for JDM and provide preliminary evidence of its validity. Methods. The Juvenile DermatoMyositis Activity Index (JDMAI) is composed of four items: physician's global assessment of overall disease activity; parent's/child's global assessment of child's wellbeing; measurement of muscle strength; and assessment of skin disease activity. The score of the JDMAI is the arithmetic sum of the scores of each individual component. Six versions of the JDMAI were tested, which differed in the tools used to assess the third and fourth items. Validation procedures were conducted using three large multinational patient samples including a total of 627 patients. Results. The JDMAI was found to possess face and content validity, good construct validity, satisfactory internal consistency (Cronbach's alpha = 0.58-0.89), fair responsiveness to clinically important change (standardized response mean = 0.82-3.12 among patients improved) and strong capacity to discriminate patients judged as being in the state of inactive disease or low, moderate or high disease activity by the physician (P<0.001) or whose parents were satisfied or not satisfied with the course of their child's illness (P<0.001). Overall, the six versions of the JDMAI showed similar metrological performances in validation analyses. Conclusion. The JDMAI was found to possess good measurement properties in a large population of patients with a wide range of disease activity, and is, therefore, suitable for use in both clinical and research settings. The final version of the JDMAI will be selected after its prospective validation.

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