Τίτλος:
Favorable outcomes with reduced steroid use in juvenile dermatomyositis
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
Background High-intensity glucocorticoid regimens are commonly used to
induce and maintain remission in Juvenile Dermatomyositis but are
associated with several adverse side-effects. Evidence-based treatment
guidelines from North American and European pediatric rheumatology
research societies both advocate induction with intravenous pulse
steroids followed by high dose oral steroids (2 mg/kg/day), which are
then tapered. This study reports 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
diagnosed with Juvenile Dermatomyositis 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, (IQR 3-7.5). Myositis control
was achieved in a median of 7.1 months (IQR 0.9-63.4). Cutaneous disease
control was achieved in a median of 16.7 months (IQR 4.3-89.5). The
median starting dose of glucocorticoids was 0.85 mg/kg/day, (IQR
0.5-1.74). The median duration of steroid treatment was 9.1 months, (IQR
4.7-17.4), while the median duration of any pharmacotherapy was 29.2
months (IQR 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 Juvenile Dermatomyositis include oral
glucocorticoids beginning at 2 mg/kg/day and reduced over a prolonged
time period. However, our results suggest that treatment using reduced
doses and duration with early use of steroid-sparing agents is
comparably effective in achieving favorable outcomes in Juvenile
Dermatomyositis.
Συγγραφείς:
Orandi, Amir B.
Fotis, Lampros
Lai, Jamie
Morris, Hallie and
White, Andrew J.
French, Anthony R.
Baszis, Kevin W.
Περιοδικό:
Pediatric Rheumatology
Λέξεις-κλειδιά:
Juvenile dermatomyositis; Calcinosis; Biologic therapy; Glucocorticoids;
Pediatric rheumatology
DOI:
10.1186/s12969-021-00615-0
