Corticosteroid discontinuation, complete clinical response and remission in juvenile dermatomyositis

with the Childhood Myositis Heterogeneity Study Group , Anna Jansen

Research output: Contribution to journalArticle

Abstract

OBJECTIVE: A North American registry of JDM patients was examined for frequency of and factors associated with corticosteroid discontinuation, complete clinical response and remission.

METHODS: We evaluated probability of achieving final corticosteroid discontinuation, complete clinical response and remission in 307 JDM patients by Weibull time-to-event modelling; conditional probability of complete clinical response and remission using Bayesian network modelling; and significant predictors with multivariable Markov chain Monte-Carlo Weibull extension models.

RESULTS: The probability of corticosteroid discontinuation was 56%, complete clinical response 38% and remission 30% by 60 months after initial treatment in 105 patients. The probability of remission was conditional on corticosteroid discontinuation and complete clinical response. Photosensitivity, contractures and a longer time to complete clinical response were predictive of the time to final corticosteroid discontinuation. Anti-MJ (NXP2) autoantibodies and a Northwest residential geoclimatic zone were predictive of shorter time to complete clinical response, while dysphonia, contractures, an increase in medications within 24 months and a longer time to corticosteroid discontinuation were associated with longer time to complete clinical response. Anti-p155/140 (TIF1) autoantibodies, an increase in medications within 12-24 months, or longer times to corticosteroid discontinuation and complete clinical response were associated with longer time to remission.

CONCLUSION: JDM patients achieve favourable outcomes, including corticosteroid discontinuation, complete clinical response and remission, although timelines for these may be several years based on time-dependent analyses. These outcomes are inter-related and strong predictors of each other. Selected clinical features and myositis autoantibodies are additionally associated with these outcomes.

Original languageEnglish
Pages (from-to)2134-2145
Number of pages12
JournalRheumatology (Oxford, England)
Volume60
Issue number5
Early online date2020
DOIs
Publication statusPublished - 1 May 2021

Bibliographical note

Published by Oxford University Press on behalf of the British Society for Rheumatology 2020. This work is written by US Government employees and is in the public domain in the US.

Keywords

  • complete clinical response
  • corticosteroid discontinuation
  • juvenile dermatomyositis
  • myositis autoantibodies
  • outcomes
  • remission

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