Juvenile dermatomyositis treated with methylprednisolone repeated intermittent pulse combined with mycophenolate mofetil: A retrospective case series study

Abstract Background: This study aimed to investigate the clinical effectivity of intravenous methylprednisolone repeated intermittent pulse combined with mycophenolate mofetil in the treatment of newly diagnosed juvenile dermatomyositis and to review the literatures. Methods: Reviewed the clinical data of 12 juvenile dermatomyositis patients, from January 2014 to January 2017, hospitalized and treated with intravenous methylprednisolone repeated intermittent pulse combined with mycophenolate mofetil. Results: A total of 12 patients were enrolled, and their overall treatment response rate was 100%. After treatment, all patients showed significant improvement in cutaneous performance and children myositis assessment scores. The median recovery time of muscle enzyme and children myositis assessment scores were 1.8 months and 19.6 months, respectively. During the intravenous methylprednisolone repeated intermittent pulse treatment, the body weight percentile was higher than that before treatment, blood pressure and intraocular pressure were transiently increased, and the level of serum lipids, glucose and calcium showed no significant changes. During the maintenance treatment period, the height percentile was increased when compared to those before treatment, while the weight percentile, blood pressure and intraocular pressure of the patients showed no significant changes. Two patients with recurrence were followed up for more than 3 years after corticosteroid re-withdrawal, and no relapse was seen till now. Conclusion: Intravenous methylprednisolone repeated intermittent pulse combined with mycophenolate mofetil showed positive outcomes and good safety index in juvenile dermatomyositis patients. It is likely considered to be a promising treatment strategy for juvenile dermatomyositis. Trial registration: ClinicalTnals, ID：NCT05509140. Registered 18 August 2022 - Retrospectively registered, https://register.clinicaltrials.gov/prs/app/action/LogoutUser?uid=U00052MF&ts=12&cx=mcg5gj.