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


CONCLUSIONS
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.
Fisler
Rita E
RE
Dermatology and Rheumatology Programs, Children's Hospital, Harvard Medical School, Boston, MA 02115, USA.
Liang
Marilyn G
MG
Fuhlbrigge
Robert C
RC
Yalcindag
Ali
A
Sundel
Robert P
RP
D002114:Calcinosis / Q000209:etiology* / Q000517:prevention & control; D002648:Child; D002675:Child, Preschool; D003882:Dermatomyositis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D011379:Prognosis; D016896:Treatment Outcome
D005938:Glucocorticoids; D011241:Prednisone; D008727:Methotrexate