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

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.

• 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)

Chemical References

• Glucocorticoids
• Prednisone
• Methotrexate

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