Abatacept, a selective modulator of the CD80/86-CD28 costimulatory signal was successfully used in patients with non-severe GPA and is currently in a phase III trial (ABROGATE, NCT02108860), which suggests that the CTLA-4 protein might be of importance in AAV pathogenesis [31]. This evidence concerns the gene CD28 and granulomatosis with polyangiitis.