Achieving Medication‐Free Remission in Patients With Juvenile Dermatomyositis

Objective Prognostic factors associated with medication discontinuation in children with juvenile dermatomyositis (JDM) remain largely elusive. We aim to identify the predictors of medication‐free remission (MFR) in children with JDM. Methods In this retrospective study, patients diagnosed with JDM...

Full description

Saved in:
Bibliographic Details
Main Authors: Ilaria Maccora, Hermine I. Brunner, Amy Cassedy, Mekibib Altaye, Megan Quinlan‐Waters, Daniel J. Lovell, Alexei Grom, Sheila T. Angeles‐Han
Format: Article
Language:English
Published: Wiley 2025-01-01
Series:ACR Open Rheumatology
Online Access:https://doi.org/10.1002/acr2.11751
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1832542416007069696
author Ilaria Maccora
Hermine I. Brunner
Amy Cassedy
Mekibib Altaye
Megan Quinlan‐Waters
Daniel J. Lovell
Alexei Grom
Sheila T. Angeles‐Han
author_facet Ilaria Maccora
Hermine I. Brunner
Amy Cassedy
Mekibib Altaye
Megan Quinlan‐Waters
Daniel J. Lovell
Alexei Grom
Sheila T. Angeles‐Han
author_sort Ilaria Maccora
collection DOAJ
description Objective Prognostic factors associated with medication discontinuation in children with juvenile dermatomyositis (JDM) remain largely elusive. We aim to identify the predictors of medication‐free remission (MFR) in children with JDM. Methods In this retrospective study, patients diagnosed with JDM according to Peter & Bohan criteria and followed for ≥18 months at a tertiary care center from 2006 through 2022 were included. Data extracted included demographics, physical examination, laboratory results, and medications. MFR was defined as inactive JDM after discontinuation of all systemic immunosuppressives for ≥6 months, in line with international consensus guidelines for trials of therapies in idiopathic inflammatory myopathies. A two‐sided P < 0.05 was considered statistically significant. Results Of 55 patients with JDM (63.6% female, age median [interquartile range (IQR)] 6 [3.5–12] years), 29 (52.7%) achieved MFR after a median (IQR) of 33 (22.5–55.2) months. MFR was more common in those who were younger at JDM diagnosis (median 5 vs 8 years, P = 0.008), had early resolution of disease activity (median 11 vs 18 months, P < 0.001), and presented with Gottron papules (χ2 = 5.25; P = 0.022) and elevated lactate dehydrogenase (χ2 = 4.82, P = 0.028). Diagnosis of JDM before 5 years old (odds ratio 4.5, 95% confidence interval [CI] 1.2–16.7) was the only predictor of MFR in our multivariate model (area under the curve 0.65, 95% CI 0.53–0.76). Conclusion Half of our patients with JDM achieved MFR. Age at JDM diagnosis may be an important predictor of achieving MFR.
format Article
id doaj-art-f52a2a384b5c41469f0e048ae5c6415e
institution Kabale University
issn 2578-5745
language English
publishDate 2025-01-01
publisher Wiley
record_format Article
series ACR Open Rheumatology
spelling doaj-art-f52a2a384b5c41469f0e048ae5c6415e2025-02-04T06:21:23ZengWileyACR Open Rheumatology2578-57452025-01-0171n/an/a10.1002/acr2.11751Achieving Medication‐Free Remission in Patients With Juvenile DermatomyositisIlaria Maccora0Hermine I. Brunner1Amy Cassedy2Mekibib Altaye3Megan Quinlan‐Waters4Daniel J. Lovell5Alexei Grom6Sheila T. Angeles‐Han7Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, and University of Florence and Meyer Children's Hospital IRCCS Florence ItalyCincinnati Children's Hospital Medical Center and University of Cincinnati College of Medicine Cincinnati OhioCincinnati Children's Hospital Medical Center and University of Cincinnati College of Medicine Cincinnati OhioCincinnati Children's Hospital Medical Center and University of Cincinnati College of Medicine Cincinnati OhioCincinnati Children's Hospital Medical Center Cincinnati OhioCincinnati Children's Hospital Medical Center and University of Cincinnati College of Medicine Cincinnati OhioCincinnati Children's Hospital Medical Center and University of Cincinnati College of Medicine Cincinnati OhioCincinnati Children's Hospital Medical Center and University of Cincinnati College of Medicine Cincinnati OhioObjective Prognostic factors associated with medication discontinuation in children with juvenile dermatomyositis (JDM) remain largely elusive. We aim to identify the predictors of medication‐free remission (MFR) in children with JDM. Methods In this retrospective study, patients diagnosed with JDM according to Peter & Bohan criteria and followed for ≥18 months at a tertiary care center from 2006 through 2022 were included. Data extracted included demographics, physical examination, laboratory results, and medications. MFR was defined as inactive JDM after discontinuation of all systemic immunosuppressives for ≥6 months, in line with international consensus guidelines for trials of therapies in idiopathic inflammatory myopathies. A two‐sided P < 0.05 was considered statistically significant. Results Of 55 patients with JDM (63.6% female, age median [interquartile range (IQR)] 6 [3.5–12] years), 29 (52.7%) achieved MFR after a median (IQR) of 33 (22.5–55.2) months. MFR was more common in those who were younger at JDM diagnosis (median 5 vs 8 years, P = 0.008), had early resolution of disease activity (median 11 vs 18 months, P < 0.001), and presented with Gottron papules (χ2 = 5.25; P = 0.022) and elevated lactate dehydrogenase (χ2 = 4.82, P = 0.028). Diagnosis of JDM before 5 years old (odds ratio 4.5, 95% confidence interval [CI] 1.2–16.7) was the only predictor of MFR in our multivariate model (area under the curve 0.65, 95% CI 0.53–0.76). Conclusion Half of our patients with JDM achieved MFR. Age at JDM diagnosis may be an important predictor of achieving MFR.https://doi.org/10.1002/acr2.11751
spellingShingle Ilaria Maccora
Hermine I. Brunner
Amy Cassedy
Mekibib Altaye
Megan Quinlan‐Waters
Daniel J. Lovell
Alexei Grom
Sheila T. Angeles‐Han
Achieving Medication‐Free Remission in Patients With Juvenile Dermatomyositis
ACR Open Rheumatology
title Achieving Medication‐Free Remission in Patients With Juvenile Dermatomyositis
title_full Achieving Medication‐Free Remission in Patients With Juvenile Dermatomyositis
title_fullStr Achieving Medication‐Free Remission in Patients With Juvenile Dermatomyositis
title_full_unstemmed Achieving Medication‐Free Remission in Patients With Juvenile Dermatomyositis
title_short Achieving Medication‐Free Remission in Patients With Juvenile Dermatomyositis
title_sort achieving medication free remission in patients with juvenile dermatomyositis
url https://doi.org/10.1002/acr2.11751
work_keys_str_mv AT ilariamaccora achievingmedicationfreeremissioninpatientswithjuveniledermatomyositis
AT hermineibrunner achievingmedicationfreeremissioninpatientswithjuveniledermatomyositis
AT amycassedy achievingmedicationfreeremissioninpatientswithjuveniledermatomyositis
AT mekibibaltaye achievingmedicationfreeremissioninpatientswithjuveniledermatomyositis
AT meganquinlanwaters achievingmedicationfreeremissioninpatientswithjuveniledermatomyositis
AT danieljlovell achievingmedicationfreeremissioninpatientswithjuveniledermatomyositis
AT alexeigrom achievingmedicationfreeremissioninpatientswithjuveniledermatomyositis
AT sheilatangeleshan achievingmedicationfreeremissioninpatientswithjuveniledermatomyositis