Favorable outcomes with reduced steroid use in juvenile dermatomyositis

Pediatric Rheumatology

Jamie Lai¹  Hallie Morris²  Kevin W. Baszis³  Anthony R. French³  Andrew J. White³  Amir B. Orandi⁴  Lampros Fotis⁵ 

[1] Department of Pediatrics, Washington University School of Medicine, St. Louis, MO, USA;Present Address: Department of Pediatrics, Division of Pediatric Rheumatology, Baylor College of Medicine, Houston, TX, USA;Department of Pediatrics, Washington University School of Medicine, St. Louis, MO, USA;Present Address: Division of Neonatology, George Washington University School of Medicine and Health Science, Washington, D.C, USA;Division of Pediatric Rheumatology/Immunology, Washington University of School of Medicine, St Louis, MO, USA;Division of Pediatric Rheumatology/Immunology, Washington University of School of Medicine, St Louis, MO, USA;Present Address: Department of Pediatric and Adolescent Medicine, Division of Pediatric Rheumatology, Mayo Clinic, Rochester, MN, USA;Division of Pediatric Rheumatology/Immunology, Washington University of School of Medicine, St Louis, MO, USA;Present Address: Department of Pediatrics, Division of Pediatric Rheumatology, National and Kapodistrian University of Athens, Athens, Greece;

关键词: Juvenile dermatomyositis;    Calcinosis;    Biologic therapy;    Glucocorticoids;    Pediatric rheumatology;   

DOI  :  10.1186/s12969-021-00615-0

来源: Springer

【 摘 要 】

BackgroundHigh-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.MethodsWe 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.ResultsOf 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%).ConclusionCurrent 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.

【 授权许可】

CC BY