Objectives: To describe a Romanian cohort of patients with juvenile dermatomyositis (JDM) and to identify factors associated with disease severity, complete clinical response, and sustained remission.
Methods: We retrospectively reviewed data from 30 JDM patients from 2013 to 2022. The inactive disease state was defined as no active skin rash, muscle weakness, or elevated muscle enzymes. A complete clinical response implied a status of inactive disease maintained for six consecutive months while on medication and remission of inactive disease for at least six consecutive months after treatment. Association factors and predictors of time to complete clinical response and time to remission emerged from bivariate correlation (Pearson's coefficient) and univariate survival analysis (Kaplan-Meier analysis).
Results: The median times to complete clinical response and time to remission for the entire cohort were 30.5 months (2.5 years) and 48.5 months (4.04 years), respectively. Nine patients (30%) had a severe disease course, while twenty-one patients (70%) had a mild/moderate course. The presence of calcinosis, time to corticosteroid discontinuation, history of treatment escalation in the first 18 months, and treatment with azathioprine or biologic DMARDs were strongly associated with a longer time to clinical remission (Pearson's > 0.5, p < 0.05). Seven patients (23%) achieved remission, and none of them relapsed during the subsequent median follow-up of 19 months.
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http://dx.doi.org/10.1097/RHU.0000000000002092 | DOI Listing |
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