Consistently with a central role of pDC in SLE, a recent phase 2 therapeutic trial assessing a monoclonal Ab ligating BDCA2 that inhibits the production of IFN-I and other inflammatory mediators has shown efficacy in reducing skin lesions and IFN signature in the blood (Furie et al., 2019a). This evidence concerns the gene CLEC4C and systemic lupus erythematosus.