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J Am Acad Dermatol. 2002 Oct;47(4):505-11.

Aggressive management of juvenile dermatomyositis results in improved outcome and decreased incidence of calcinosis.

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  • 1Dermatology and Rheumatology Programs, Children's Hospital, Harvard Medical School, Boston, MA 02115, USA.

Abstract

BACKGROUND:

Long-term consequences of juvenile dermatomyositis (JDM) include onset of calcinosis and subsequent functional impairment. Historic incidence of calcinosis has been reported between approximately 23% and 70%. Recent reports note improved outcome with high-dose steroids, yet the incidence of calcinosis has remained above 30%.

OBJECTIVE:

We attempted to determine whether rapid, aggressive disease management can prevent calcinosis and improve functional outcome.

METHODS:

Medical records of children with JDM managed at a pediatric medical center during a 10-year period were reviewed to determine (1) interval between onset of symptoms and diagnosis, (2) treatment modality, and (3) functional outcome and presence of calcinosis.

RESULTS:

A total of 21 female and 14 male subjects diagnosed with JDM met inclusion criteria, with a mean age of diagnosis of 7.6 +/- 3.9 years. Mean time from onset of symptoms to treatment was 6.6 +/- 8.2 months. Pulse intravenous methylprednisone (30 mg/kg daily) or high-dose prednisone was used in 31 of 35 patients. Patients who failed to respond within 6 weeks were started on a regimen of methotrexate (23/35). At follow-up, 5 patients had mild calcinosis (14%). Onset of calcinosis was associated with a longer time to diagnosis and treatment (30.6 vs 6 months, P =.003), a longer duration of elevated muscle enzymes (34 vs 12.6 months, P =.03), and longer disease duration (42.8 vs 22.2 months, P =.05).

CONCLUSION:

Stepwise, aggressive treatment directed at achieving rapid and complete control of muscle inflammation is highly successful in minimizing the long range sequelae of JDM, including calcinosis.

PMID:
12271292
[PubMed - indexed for MEDLINE]
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