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  • Poster presentation
  • Open Access

8.3 Disease patterns in Danish Juvenile Dermatomyositis patients

  • 2,
  • 1,
  • 3 and
  • 1
Pediatric Rheumatology20086 (Suppl 1) :S17

https://doi.org/10.1186/1546-0096-6-S1-S17

  • Published:

Keywords

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

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

Symptom

% of patients

Proximal muscle weakness

81

Fatigue

74

Erythema

74

Gottrons papules

70

Heliotrope

57

Arthralgia

41

Fever

32

Arthritis

30

Weight loss

30

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.

Authors’ Affiliations

(1)
Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
(2)
Holbaek County University Hospital, Holbaek, Denmark
(3)
Skejby University Hospital, Aarhus, Denmark

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