Reply to ‘Inborn errors of immunity and AAV: a complex picture’

We thank Clément Triaille, Marie-Louise Frémond and Augusto Vaglio for their interest in our Review (Gül, A et al. The pathogenesis, clinical presentations and treatment of monogenic systemic vasculitis. Nat. Rev. Rheumatol. 21, 414–425 (2025))¹, and for providing additional examples of monogenic disorders that can be associated with the development of anti-neutrophil cytoplasm antibodies (ANCA) and vasculitic manifestations (Triaille, C., Frémond, M. L. & Vaglio, A. Inborn errors of immunity and AAV: a complex picture. Nat. Rev. Rheumatol. https://doi.org/10.1038/s41584-025-01299-6 (2025))².

In our Review¹, we focused on monogenic autoinflammatory diseases that present with findings of vasculitis involving blood vessels of all sizes and types. The pathogenic mechanisms underlying these conditions are complex and include the activation of myeloid cells, type I interferon-driven autoimmune responses, dysregulated adaptive immunity, endothelial dysfunction and injury and thrombotic vasculopathies.