Anti-LAMP-2 antibodies were firstly described in 87% of patients with RLV and this high prevalence of anti-LAMP-2 antibodies was further confirmed in a multicenter study that included patients with ANCA-associated vasculitis (i.e., GPA, MPA, and RLV) at disease presentation from Austria, the Netherlands, and from the United Kingdom with a positivity of 89%, 91%, and 80%, respectively. Here, LAMP2 is linked to granulomatosis with polyangiitis.