Type I interferon (IFN) plays an important role in the pathogenesis of systemic lupus erythematosus (SLE) and cutaneous manifestations. Anifrolumab, a fully humanized monoclonal antibody against type 1 IFN receptor, has shown a significant reduction of the disease activity of SLE and cutaneous manifestations in adults. We reported two pediatric SLE patients with refractory cutaneous lesions that dramatically improved after anifrolumab therapy. This is the first study to show clinical effects of anifrolumab for pediatric SLE patients. These cases suggest the efficacy of anifrolumab for pediatric SLE patients with refractory cutaneous lesions and elevated IFN levels.
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