Juvenile dermatomyositis at diagnosis: Clinical characteristics of 79 children

Lauren M. Pachman, Jennifer R. Hayford, Ahn Chung, Claire A. Daugherty, Mark A. Pallansch, Chester W. Fink, Harry L. Gewanter, Rita Jerath, Bianca A. Lang, James Sinacore, Ilona S. Szer, Alan R. Dyer, Marc C. Hochberg

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Abstract

Objective: To evaluate demographic and clinical characteristics, duration of time between disease onset (date of first rash and/or weakness), and diagnosis/therapy, as well as socioeconomic status, of children with newly diagnosed juvenile dermatomyositis (JDM). Structured telephone interview of families of a cohort of 79 children with JDM: interval between onset of symptoms to diagnosis, median of 3 months (range 0.5-20.0). Results: At diagnosis, all the children had rash (100%) and proximal muscle weakness (100%); 58 (73%) had muscle pain; 51 (65%) fever; 35 (44%) dysphagia; 34 (43%) hoarsehess; 29 (37%) abdominal pain; 28 (35%) arthritis; 18 (23%) calcinosis, and 10 (13%) melena. Muscle derived enzymes were normal in 10% of the children. Of the 43 children who had an electromyogram (EMG), 8 (19%) had normal results. Fifty-one children had a muscle biopsy; the results were normal/nondiagnostic in 10 (20%). Median time from disease onset to diagnosis was different between racial groups: Caucasians (n=59) 2.0 months; for minorities (n=20), 6.5 months, (p=0.0008). The median time from disease onset to therapy was: Caucasians, 3.0 months; minorities, 7.2 months (p=0.002). Report of calcinosis was associated with increased time to diagnosis and therapy (p=0.04). In the 33 children whose first symptom occurred in June- September, rash preceded or accompanied onset of muscle weakness in 83% (n=27). Ninety-one percent of the children were given steroid therapy and 9% received methotrexate as well. Conclusion: The results of an undirected site for muscle biopsy or EMG may not be diagnostic. Minority children had a longer interval between first JDM symptom and diagnosis/therapy than Caucasian children. Delay in diagnosis/therapy was associated with calcinosis.

Original languageEnglish (US)
Pages (from-to)1198-1204
Number of pages7
JournalJournal of Rheumatology
Volume25
Issue number6
Publication statusPublished - Jun 1 1998

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Keywords

  • Access to medical care
  • Clinical laboratory
  • Epidemiology
  • Juvenile dermatomyositis

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Immunology

Cite this

Pachman, L. M., Hayford, J. R., Chung, A., Daugherty, C. A., Pallansch, M. A., Fink, C. W., ... Hochberg, M. C. (1998). Juvenile dermatomyositis at diagnosis: Clinical characteristics of 79 children. Journal of Rheumatology, 25(6), 1198-1204.