Anifrolumab Therapy in Dermatomyositis: New Insights From Refractory Patients

OBJECTIVE: To analyze the efficacy and safety of anifrolumab, a monoclonal antibody targeting the type I interferon receptor subunit 1, as a therapeutic option in patients with refractory cutaneous dermatomyositis (DM). METHODS: Patients with DM presenting with cutaneous involvement refractory to different immunosuppressive treatments were enrolled. The Cutaneous Dermatomyositis Disease Area and Severity Index activity (CDASI-A) was used to evaluate the evolution of skin involvement. Manual muscle test-8 (MMT-8) and patient global assessment (PtGA) scores were collected, to evaluate both muscle involvement and patients' quality of life. We registered the changes in glucocorticoid (GC) daily dose, to explore the possible GC-sparing effect of the drug. RESULTS: We enrolled 4 patients (50% female, mean age 60 years). At enrollment, they showed an active cutaneous disease (mean CDASI-A 27/100), mean MMT-8 of 67.5/80, mean daily dose of GC of 10 mg of prednisone, and mean PtGA value of 7.5/10. After treatment, all these variables improved, with mean CDASI-A decreasing to 8.75/100, MMT-8 increasing to 71.3/80, daily GC dose decreasing to 4 mg, and PtGA decreasing to 1.5/10. CONCLUSION: The data we collected showed a significant reduction in CDASI-A values. Further, our results showed an improvement in muscle involvement and in patients' perception of disease burden. Finally, a GC-sparing effect and a good global safety profile of anifrolumab were observed, thus confirming anifrolumab as a new valid therapeutic option in patients with refractory cutaneous DM.