Achieving Medication-Free Remission in Patients With Juvenile Dermatomyositis

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.

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.

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).

Half of our patients with JDM achieved MFR. Age at JDM diagnosis may be an important predictor of achieving MFR.