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Duration of illness is an important variable for untreated children with juvenile dermatomyositis
Lauren M Pachman1, Kathy Abbott, James M Sinacore
1Department of Pediatrics, Division of Immunology/Rheumatology, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA. pachman@#northwestern.edu
Insights
The duration of untreated juvenile dermatomyositis (JDM) impacts disease severity and treatment decisions. Longer symptom duration in JDM patients may affect muscle enzyme levels and calcifications.
Area of Science:
- Pediatric Rheumatology
- Autoimmune Diseases
- Clinical Research
Background:
- Juvenile dermatomyositis (JDM) is a rare autoimmune disease affecting children.
- Understanding factors influencing JDM presentation is crucial for effective management.
Purpose of the Study:
- To assess how the length of time before diagnosis and treatment in children with JDM affects their condition.
- To identify correlations between symptom duration and clinical/laboratory findings at diagnosis.
Main Methods:
- Analysis of physical and laboratory data from 166 untreated children with JDM at their initial physician visit.
- Use of disease activity scores (DASs) for skin and muscle involvement.
- Comparison of patient height and weight with national data.
Main Results:
- Children with JDM were shorter and lighter than national averages.
- Nonwhite children exhibited greater weakness compared to white children.
- Longer untreated JDM duration correlated with increased muscle weakness and pathological calcifications, and tended toward normal muscle enzyme levels.
Conclusions:
- The duration of untreated symptoms is a significant factor in JDM.
- This variable should inform diagnostic criteria and treatment intensity for JDM in children.
Objective:
To evaluate the impact of duration of untreated symptoms in children with juvenile dermatomyositis (JDM) on clinical and laboratory status at diagnosis.
Study Design:
We examined physical and laboratory data from the first physician visit for 166 untreated children with JDM. Disease activity scores (DASs) assessed skin and muscle involvement. Height and weight were compared with the National Health and Nutrition Examination Survey III dataset. Duration of untreated illness was designated as the time from first sign of rash or weakness to diagnostic visit.
Results:
Boys and girls with untreated JDM were shorter and lighter than national norms (P > .0005 for both), and nonwhite children were weaker than white children (P > .0005). Older children had more dysphagia (P = .017) and arthritis (P > .001). Duration of untreated JDM was negatively associated with DAS weakness (P > .0005), unrelated to DAS skin, and positively associated with pathological calcifications (P = .006). With untreated disease > or = 4.7 months, serum levels of 4 muscle enzymes (aldolase, lactic dehydrogenase, creatine kinase, serum glutamic-oxaloacetic transaminase/aspartate aminotransferase) tended toward normal (P > .01 for each).
Conclusions:
Duration of untreated symptoms is an important variable and should be included in decisions concerning both diagnostic criteria and intensity of therapy for children with JDM.
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