Favorable outcomes with reduced steroid use in juvenile dermatomyositis

• Published in: Pediatric Rheumatology 2021-08, Vol.19 (1), p.127-127, Article 127
• Main Authors: Orandi, Amir B, Fotis, Lampros, Lai, Jamie, Morris, Hallie, White, Andrew J, French, Anthony R, Baszis, Kevin W
• Format: Article
• Language: English
• Subjects: Administration, Oral; Biologic therapy; Biological Therapy - methods; Calcinosis; Calcinosis - etiology; Calcinosis - prevention & control; Care and treatment; Child; Corticosteroids; Dermatomyositis; Dermatomyositis - blood; Dermatomyositis - diagnosis; Dermatomyositis - drug therapy; Dermatomyositis - physiopathology; Disease control; Dose-Response Relationship, Drug; Drug Tapering - methods; Duration of Therapy; Evidence-based medicine; Female; Glucocorticoids; Glucocorticoids - administration & dosage; Glucocorticoids - adverse effects; Health aspects; Humans; Immunosuppressive agents; Juvenile dermatomyositis; Laboratories; Male; Medical research; Medicine, Experimental; Mortality; Oral medication; Outcome Assessment, Health Care - methods; Outcome Assessment, Health Care - statistics & numerical data; Patients; Pediatric rheumatology; Pediatrics; Remission (Medicine); Remission Induction - methods; Rheumatology; Skin diseases; Steroids; Symptom Assessment - methods; United States - epidemiology
• ISSN: 1546-0096; 1546-0096
• DOI: 10.1186/s12969-021-00615-0

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. 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. 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%). 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.