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8.3 Disease patterns in Danish Juvenile Dermatomyositis patients

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Purpose

Retrospective assessment of disease characteristics and outcome for the Danish cohort of Juvenile Dermatomyositis (JDM) patients, 1977 – 2005.

Evaluation of the Myositis disease activity assessment tool (MYOACT) and Myositis intention to treat activity index (MITAX) as prognostic tools.

Methods

Hospital records from Danish JDM patients (1977 – 2005) were reviewed. The parameters of the MYOACT and MITAX were used for the database.

Results

53 patients were classified as JDM. The female:male ratio was 2:1, the mean age at disease onset was 7.1 years and the mean disease duration was 3.6 years. Most frequent symptoms at disease onset are displayed in Table 1.

Table 1 Most frequent symptoms at disease onset

At the 5-years follow-up 34% were in remission, 30% had ongoing disease and disease- or treatment-induced damage was present in 36%. In the total follow-up period (2–30 years) 3 patients (6%) had died, 68% were in full remission, 13% had ongoing disease and 13% had unknown status.

Conclusion

Most patients had a favourable outcome; however irreversible damage was found in 36% at 5-years follow-up

Baseline predictors of unfavourable disease outcome could not be identified

Due to frequently missing chart data MYOACT and MITAX could not be used as a scoring tool in this retrospective set-up

A clinical long-term follow-up study is warranted and now carried out by the authors.

Author information

Correspondence to PR Mathiesen.

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Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution 2.0 International License (https://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Mathiesen, P., Zak, M., Herlin, T. et al. 8.3 Disease patterns in Danish Juvenile Dermatomyositis patients. Pediatr Rheumatol 6, S17 (2008) doi:10.1186/1546-0096-6-S1-S17

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Keywords

  • Disease Onset
  • Myositis
  • Dermatomyositis
  • Unknown Status
  • Retrospective Assessment