French, Anthony R.

Abstract Background High-intensity glucocorticoid regimens are commonly used to induce and maintain remission in Juvenile Dermatomyositis (JDM) but are associated with several adverse side-effects. Eminence-based treatment guidelines from CARRA and SHARE both advocate induction of intravenous pulse steroids followed by high dose oral steroids (2 mg/kg) tapered over 12 months. This study compares the time to disease control with reduced glucocorticoid dosing. Methods We retrospectively reviewed the records at a single tertiary-care children’s hospital of patients with JDM between 2000 and 2014 who had a minimum of 2 years of follow-up. The primary outcome measure was time to control of muscle and skin disease. Additional outcome measures included glucocorticoid dosing, effect of treatment on height, frequency of calcinosis and complications from treatment. Results Of the 69 patients followed during the study period, 31 fulfilled inclusion criteria. Median length of follow-up was 4.58 years, (3–7.5). Myositis control was achieved in a median of 7.1 months (range 0.9–63.4). Cutaneous disease control was achieved in a median of 16.7 months (range 4.3–89.5). The median starting dose of glucocorticoids was 0.85 mg/kg/day, (range 0.5–1.74). The median duration of steroid treatment was 9.1 months, (range 4.7–17.4), while the median duration of any pharmacotherapy was 29.2 months (range 10.4 to 121.3). Sustained disease control off medications was achieved in 21/31 (68%) patients by the end of review. Persistent calcinosis was identified in only one patient (3%). Conclusion Current accepted treatment paradigms for JDM include oral glucocorticoids beginning at 2 mg/kg/d reduced over 12 months; however, our results suggest that treatment using reduced doses and durations with early use of steroid-sparing agents are comparably effective in achieving favorable outcomes in JDM.