BAFF and selection of autoreactive B cells

B cell activating factor (BAFF) is a crucial survival factor for transitional and mature B cells, and is a promising therapeutic target for systemic lupus erythematosus (SLE). A BAFF inhibitor, belimumab, is the first new drug in 50 years to be approved for the treatment of SLE. However, the mechanism of action of this drug is not entirely clear. In this review we will focus on the role of the BAFF–APRIL signaling pathway in the selection of autoreactive B cells, and discuss whether altered selection is the mechanism for the therapeutic efficacy of BAFF inhibition in SLE.