Refractory dermatomyositis (DM) is defined as cases that do not show improvement after initial treatment with two different immunosuppressives combined with corticosteroids with or without intravenous immunoglobulins. In recent years, few studies have reported a positive response to the use of Janus kinase inhibitors (JAK‐inhibitors) for the treatment of refractory DM. A systematic literature review was performed for articles studying the use of JAK‐inhibitors for the treatment of refractory DM. We identified 38 females and 15 males treated with JAK‐inhibitors without serious side effects. Tofacitinib was the most frequently used JAK‐inhibitor followed by ruxolitinib. Significant improvement in CDASI score, muscle strength, body weight, and skin lesions were reported in most of the studies. The duration of follow‐up ranged from 1 to 15 months without relapse. Therefore, the use of JAK‐inhibitors looks promising in the treatment of refractory DM and further high volume research may be required to validate the current concept. As only case reports and series were identified without direct comparison for review, there is a potential risk of bias. Despite these limitations, we believe that the result of this analysis allows a better understanding of treatment options for refractory DM and will help generate a hypothesis that can be further tested.
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