Janus kinase inhibitor significantly improved rash and muscle strength in juvenile dermatomyositis
Yuchuan Ding, Baozhen Huang, Yuan Wang, Jun Hou, Ying Chi, Zhixuan Zhou, Jianguo Li
Abstract
Letters Janus kinase inhibitor significantly improved rash and muscle strength in juvenile dermatomyositisJuvenile dermatomyositis (JDM) is a rare systemic autoimmune vasculopathy characterised by weakness in proximal muscles and pathognomonic skin rashes. 1 Clinically, some patients are refractory to any available treatments or became steroids dependent. 2 The adverse reactions of long-term use of steroids are severe; therefore, more effective and safer medications are urgently needed.][5] Here, we want to share the JAKi using experiences of 25 refractory JDM cases who were diagnosed and classified according to Bohan and Peter's criteria and treated between November 2017 and May 2019.Written informed consents were obtained from the guardians of all patients before starting the treatment.Among 25 cases, 44% (11/25) patients were female, the mean age of onset was 4.6±3.3years and the mean age to start add-on JAKi treatment was 7.2±4.0years.The mean disease course of the 25 JDM patients before JAKi treatment is 21.0 months (range: 14.0-36.5).All cases are refractory JDMs, including 32% (8/25) ineffective patients and 68% (17/25) glucocorticoiddependent cases.After routine treatment fails, they received JAKi for 3-18 months as an off-label use.In subsequent JAKi treatment, 28% (7/25) used tofacitinib, and 72% (18/25) used ruxolitinib.In patients of <25 kg (n=11), the initial dosage was 2.5 mg twice daily, and in patients of ≥25 kg (n=14), the initial dosage was 5 mg twice daily, and one patient required the maximum dosage of 7.5 mg twice daily.The 25 patients were followed for a median of 7.0 months (range: 3-21 months).Ninety-six per cent (24/25) had rash when on August 20, 2023 by guest.