Long-term outcome in children with juvenile dermatomyositis: A single-center study from north India.
CMAS
MMT8
Myositis Disease Activity Assessment Tool (MDAAT)
aCAT
calcinosis
lipodystrophy
Journal
International journal of rheumatic diseases
ISSN: 1756-185X
Titre abrégé: Int J Rheum Dis
Pays: England
ID NLM: 101474930
Informations de publication
Date de publication:
Mar 2020
Mar 2020
Historique:
received:
16
05
2019
revised:
30
10
2019
accepted:
08
11
2019
pubmed:
4
12
2019
medline:
12
1
2021
entrez:
4
12
2019
Statut:
ppublish
Résumé
Juvenile dermatomyositis (JDM) is the commonest childhood inflammatory myopathy. Outcome of children with JDM has improved significantly with current treatment protocols. We undertook this study to evaluate long-term outcome of these children using validated outcome measures. All children diagnosed as JDM and on follow-up for more than 2 years were eligible for enrolment. Cross-sectional examination was performed to assess signs of disease activity, damage, physical function and complications by using multiple validated outcome measures/tools. Thirty-seven patients were enrolled, 19 were male. Median duration of disease at time of enrolment was 73 months (range 24-219 months) and median duration of follow-up was 60 months (range 24-218 months). Disease course was monocyclic in two-thirds of patients. Eight children were still on therapy at the time of enrolment. On Manual Muscle Testing 8, 3 and 7 children had severe weakness and mild to moderate weakness, respectively. Neck flexors were the most commonly affected muscle group. On abbreviated Cutaneous Assessment Tool, 14 children had evidence of cutaneous activity. More than 50% had at least 1 sign of cutaneous damage, most common signs being calcinosis and lipodystrophy. Nearly two-thirds of patients had damage in at least 1 organ using Myositis Damage Index. Nine children had physical dysfunction when assessed by Child Health Assessment Questionnaire. Skin disease continued to be active in a significant proportion of patients. Features of damage, namely calcinosis and lipodystrophy, were seen in more than half. Muscle disease normalized in a large proportion of patients.
Identifiants
pubmed: 31793219
doi: 10.1111/1756-185X.13759
doi:
Types de publication
Journal Article
Observational Study
Langues
eng
Sous-ensembles de citation
IM
Pagination
392-396Informations de copyright
© 2019 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.
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