Corticosteroid Discontinuation, Complete Clinical Response and Remission in Juvenile Dermatomyositis

Overview

Journal Rheumatology (Oxford)

Publisher Oxford University Press

Specialty Rheumatology

Date 2020 Oct 17

PMID 33067611

Citations 9

Authors

Takayuki Kishi

William Warren-Hicks

Nastaran Bayat

Ira N Targoff

Adam M Huber

Michael M Ward

Lisa G Rider

Affiliations

Soon will be listed here.

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.

Citing Articles

Achieving Medication-Free Remission in Patients With Juvenile Dermatomyositis.

Maccora I, Brunner H, Cassedy A, Altaye M, Quinlan-Waters M, Lovell D ACR Open Rheumatol. 2024; 7(1):e11751.

PMID: 39707697 PMC: 11707262. DOI: 10.1002/acr2.11751.

Paraneoplastic dermatomyositis and Hodgkin's lymphoma in a 14-year-old girl: a case report and literature review.

Ling Y, Hu H, Xu X, Feng J, Li M, Li H Front Oncol. 2024; 14:1416083.

PMID: 39169942 PMC: 11335551. DOI: 10.3389/fonc.2024.1416083.

Myositis-Associated Autoantibodies in Patients With Juvenile Myositis Are Associated With Refractory Disease and Mortality.

Sherman M, Noroozi Farhadi P, Pak K, Trieu E, Sarkar K, Targoff I Arthritis Rheumatol. 2024; 76(6):963-972.

PMID: 38272842 PMC: 11136598. DOI: 10.1002/art.42813.

Clinical Features and Immunogenetic Risk Factors Associated With Additional Autoantibodies in Anti-Transcriptional Intermediary Factor 1γ Juvenile-Onset Dermatomyositis.

Sherman M, Yang Q, Gutierrez-Alamillo L, Pak K, Flegel W, Mammen A Arthritis Rheumatol. 2023; 76(4):631-637.

PMID: 38059274 PMC: 10965375. DOI: 10.1002/art.42768.

Juvenile idiopathic inflammatory myositis: an update on pathophysiology and clinical care.

Papadopoulou C, Chew C, Wilkinson M, McCann L, Wedderburn L Nat Rev Rheumatol. 2023; 19(6):343-362.

PMID: 37188756 PMC: 10184643. DOI: 10.1038/s41584-023-00967-9.

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