Janus kinase inhibitors improve outcomes in refractory juvenile dermatomyositis


  • Heather Mason
  • Univadis Medical News
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A study conducted in patients with refractory juvenile dermatomyositis (JDM) showed that the use of janus kinase inhibitors (JAKi) improved refractory rash and muscle involvement, according to an article published in Annals of the Rheumatic Diseases.

The analysis included 25 patients with JDM who received JAKi off-label for three to 18 months. All cases were refractory, including eight ineffective patients and 17 glucocorticoid-dependent cases. The patients were followed for a median of seven months.

The results show that 96 per cent of patients had rash when JAKi was added, and all showed improved rashes, including cases of complete resolution. No clinically observable rash could be seen after 12 weeks of JAKi. Up to the last follow-up, 28 per cent of patients had discontinued glucocorticoids.

After treatment with JAKi, seven cases improved in childhood myositis assessment scale (CMAS) score. Two patients did not change in CMAS score but reported improvement in fatigue and activity tolerance.

These findings suggest that JAKi improved refractory rash and muscle involvement and helped to reduce or stop glucocorticoid use, without obvious side effects. The authors concluded that JAKi might be an ideal choice in children with refractory JDM.