In addition, the finding that remote polymorphisms potentially present in PR3 or remote protein–ligand interactions can lead to the activation and accessibility of a latent epitope recognized by an anti-PR3 antibody derived from a GPA patient [157] points towards high levels of complexity in terms of the relationships between autoantigens and ANCA in GPA and AAV. The gene discussed is PRTN3; the disease is granulomatosis with polyangiitis.