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.
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: Mark F Hoeltzel; Edward J Oberle; Angela Byun Robinson; Arunima Agarwal; Lisa G Rider Journal: Curr Rheumatol Rep Date: 2014-12 Impact factor: 4.592
Authors: H Gunawardena; L R Wedderburn; H Chinoy; Z E Betteridge; J North; W E R Ollier; R G Cooper; C V Oddis; A V Ramanan; J E Davidson; N J McHugh Journal: Arthritis Rheum Date: 2009-06