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.
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Authors | Rita E Fisler, Marilyn G Liang, Robert C Fuhlbrigge, Ali Yalcindag, Robert P Sundel |
Journal | Journal of the American Academy of Dermatology
(J Am Acad Dermatol)
Vol. 47
Issue 4
Pg. 505-11
(Oct 2002)
ISSN: 0190-9622 [Print] United States |
PMID | 12271292
(Publication Type: Journal Article)
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Chemical References |
- Glucocorticoids
- Prednisone
- Methotrexate
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Topics |
- Calcinosis
(etiology, prevention & control)
- Child
- Child, Preschool
- Dermatomyositis
(complications, diagnosis, drug therapy)
- Female
- Glucocorticoids
(therapeutic use)
- Humans
- Male
- Methotrexate
(therapeutic use)
- Prednisone
(therapeutic use)
- Prognosis
- Treatment Outcome
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