Inborn errors of immunity and AAV: a complex picture

We read with great interest the manuscript by Gül et al. on monogenic systemic vasculitis (Gül, A. et al. The pathogenesis, clinical presentations and treatment of monogenic systemic vasculitis. Nat. Rev. Rheumatol. 21, 414–425 (2025))1. We thank the authors for presenting a detailed synthesis of current knowledge on the clinical features of these genetic disorders and the putative mechanisms that link different genetic defects to vascular inflammation. Gül et al.1 also discuss the potential relevance of these genes (and their associated pathways) for the sporadic forms of vasculitis that they clinically resemble. We would like to provide further comments on the genetic defects associated with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV).