Key result
Myalgia, dysphonia, and cutaneous symptoms are linked to worse disease activity in juvenile dermatomyositis.
Why the study?
It is currently impossible to predict the prognosis of patients with juvenile dermatomyositis, motivating identification of clinical features associated with disease activity.
Cohort (n=340)
Yes
Specific clinical signs like myalgia and dysphonia are strongly associated with worse disease activity in JDM, and PGA provides unique information compared to other activity scores.
Answers are grounded in this paper and cite their sources.
Certain signs/symptoms were associated with JDM activity; leaves open whether they enable reliable prognostic stratification or tailor-made therapy.
BACKGROUND: It is currently impossible to predict the prognosis of patients with juvenile dermatomyositis (JDM). The aim of this study was to find clinical features most strongly associated with outcome variables in JDM as a first step towards tailor-made treatment. METHODS: In a large, prospectively followed, multicenter cohort study of 340 patients with JDM, each contributing multiple visits, a Bayesian model of disease activity was developed, using the four continuous outcome variables creatine kinase (CK), childhood myositis assessment score (CMAS), manual muscle testing of 8 muscle groups (MMT8) and the physician's global assessment of disease activity (PGA). Covariates were clinical signs and symptoms. Correlations among visits of the same patient were resolved by introducing subject-specific random effects. RESULTS: Myalgia and dysphonia were associated with worse disease activity according to all outcome variables. Periorbital rash, rash on the trunk, rash over large joints, nail fold changes and facial swelling were associated with higher PGA. Notably, periorbital rash was also associated with higher CK and lower CMAS and nail fold changes with lower CMAS. Contractures were associated with lower CMAS and MMT8 and higher PGA. Patients with higher CMAS exhibited a higher MMT8 as well. PGA had the highest probability among the four outcome variables of being abnormal even if the other three outcome variables were normal. CONCLUSIONS: The signs and symptoms associated with disease activity could be used to stratify patients and adapt treatment plans to disease activity. The correlation between CMAS and MMT8 and the unique information captured by PGA implied that PGA should be maintained as an outcome variable, whereas CMAS and MMT8 might be simplified.
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Dijkhuizen et al. (2018) conducted a cohort in Juvenile dermatomyositis (n=340). Clinical signs and symptoms (e.g., myalgia, dysphonia, cutaneous manifestations) vs. Absence of specific clinical signs and symptoms was evaluated on Disease activity measured by a joint model of creatine kinase (CK), childhood myositis assessment score (CMAS), manual muscle testing of 8 muscle groups (MMT8), and physician's global assessment (PGA). Myalgia, dysphonia, and cutaneous symptoms were associated with worse disease activity in juvenile dermatomyositis, and the physician's global assessment had a 52% probability of remaining abnormal when other parameters were normal.
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