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8.3 Disease patterns in Danish Juvenile Dermatomyositis patients
Pediatric Rheumatologyvolume 6, Article number: S17 (2008)
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.
Hospital records from Danish JDM patients (1977 – 2005) were reviewed. The parameters of the MYOACT and MITAX were used for the database.
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.
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.
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.