JAK‐inhibitors for dermatomyositis: A concise literature review

Abstract

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.

A glance into the future of myositis therapy

“…24 Clinical data on the effects of JAK inhibitors in myositis is scanty. Tofacitinib has been more extensively studied than baricitinib, and has been shown to have efficacy especially on the skin manifestations of DM and possibly on arthritis and muscle involvement, 25,26 while a Japanese study suggested its utility as add-on therapy in severe ILD in patients with anti-MDA5+ DM. 27 In our experience, tofacitinib has proved useful in significantly ameliorating skin DM manifestations, including calcifications, 28 in patients that had been resistant to other drugs.…”

Targeting intracellular pathways in idiopathic inflammatory myopathies: A narrative review

“…In the last few years, an impressive number of reports have highlighted Tofacitinib efficacy in diverse domains of DM patients with heterogeneous phenotypes ( 65 ).…”

Diagnosis and Management of Cutaneous Manifestations of Autoimmune Connective Tissue Diseases

“… 99 Most studies to date exploring JAK inhibitor use in DM administered the drug as an adjuvant to other systemic therapies. 96 …”

“…93 Janus kinase (JAK) inhibitors have shown increasing evidence as an effective therapy in both CDM and CADM. 96,97 In the largest prospective clinical trial using tofacitinib monotherapy in ten patients with DM, the skin disease activity as measured by the CDASI improved from moderate to severe skin disease activity to mild in 70% of patients. 98 In another prospective study with 18 DM patients studying tofacitinib with concomitant glucocorticoid therapy versus conventional therapy, the former cohort exhibited a higher survival rate at 6 months as well as improved lung function.…”

“…99 Most studies to date exploring JAK inhibitor use in DM administered the drug as an adjuvant to other systemic therapies. 96 Multiple trials for novel medications are currently underway, with a focus on mitigating the inflammatory pathway leading to disease. Apremilast is a phosphodiesterase 4 inhibitor which was recently shown to improve skin symptoms in a small phase 1b clinical trial for patients with refractory DM.…”