Elsevier

The Journal of Pediatrics

Volume 148, Issue 2, February 2006, Pages 247-253
The Journal of Pediatrics

Original article
Duration of illness is an important variable for untreated children with juvenile dermatomyositis

https://doi.org/10.1016/j.jpeds.2005.10.032Get rights and content

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 ≥ 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.

Section snippets

Patient Recruitment

Newly diagnosed children with JDM living in the continental United States were recruited during a 5-year period (1994 to 1999). The JDM Research Registry hotline gave both parents and physicians information about the study, which was publicized at scientific and medical meetings and in medical journals. Registry participation in the last 2 years of the study (1998 and 1999) was facilitated by a website describing the purpose of the study and providing access to the consent form required for

Untreated Children With Newly Diagnosed JDM

A review of the medical records from the 323 enrolled candidates identified 166 children who had not received any steroid or other immunosuppressive agent, including hydroxychloroquine, before the diagnostic testing. Demographic characteristics of this group were comparable to those of the previously described larger group of 323 who enrolled in the NIAMS Registry (Table I),3 as well as the group who had received medical treatment for symptoms before the diagnosis of JDM. The untreated group of

Discussion

Several previous studies have described the symptoms of children with JDM at diagnosis,9, 10, 11, 12 but the patient groups were typically small, and data on duration of untreated disease were not available. The mean age at diagnosis (7.5 years) and sex ratio (2 girls to 1 boy) of the children in our study are similar to those in other published reports12 and also to the data from the Pediatric Rheumatology International Trials Organization (PRINTO) groups, which studied the onset of JDM in 295

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    Supported by the National Institute of Arthritis, Muscle and Skin Diseases (NIAMS) (grant NO1-AR-4-2219) (L.M.P.), the Juvenile Dermatomyositis Research Registry (L.M.P.), the Arthritis Foundation (L.M.P), the Marlene Apfelbaum Foundation (L.M.P.), the Pappas Foundation (L.M.P.), P60 AR 30692 (L.M.P. and R.R.G.), and the National Institutes of Health/NIAMS (grant K24 02138) (R.R.G.).

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