Pulse Steroids and Mycophenolate in Juvenile Dermatomyositis Save
JAMA Dermatology has published a pilot study demonstrating the safety and efficacy of intermittent intravenous methylprednisolone pulse (IVMP) therapy plus mycophenolate in 28 patients with juvenile dermatomyositis (JDM).
While most believe that first line therapy in JCM is oral glucocorticoids (GCs) with methotrexate, some have advocated for the advantages of IVMP and mycophenolate mofetil (MMF) as an alternative. This single center reports their results in treating JDM with intermittent IVMP plus MMF as first-line therapy.
A total of 28 JDM patients meeting 2006 Bohan/Peter criteria or 2017 EULAR/ACR classification criteria were treated and followed for 4 or more years. IVMP was given as 20-30 mg/kg/d for 3 consecutive days per course (for 7 to 9 courses) plus MMF (500-600 mg/m2 twice daily). Hydroxychloroquine was added for skin disease activity scores (DASs >4 points in 17 of 28 patients and intravenous immunoglobulin was added for poor muscle strength or rash after 3 to 4 IVMP courses in 9 of 28 patients. Outcomes included the Childhood Myositis Assessment Scale (CMAS) scores, and the validated 20-point DAS.
The median age at diagnosis was 7.0 years, and the median follow-up was 61 months.
The median oral prednisone dose was 7.5 mg qd during IVMP intervals, and the median time to discontinue GCs was 23.5 months and to discontinue all medications was 29.5 months.
Overall, 26 patients (93%) achieved complete clinical response (after 10 mos) and clinical remission (after 35.5 mos). Muscle enzymes normalized after a median of 3 months; CMAS scores and DAS scores improved. Only 5 patients (18%) relapsed; none had calcinosis or interstitial lung disease.
Major infections occurred at 2.3 per 100 person-years (no intensive care unit admissions or deaths). Six patients had transient steroid-induced ocular hypertension, 4 with transient glaucoma; 1 had cataracts; and no osteonecrosis or fractures occurred.
These results suggests that the regimen of IVMP and MMF, is a therapeutic option for JDM. These results are confounded by the use of IVIG and HCG in 33%-60% of JDM patients.
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The author has no conflicts of interest to disclose related to this subject
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