IBD emerged as a hallmark of certain PIRDs, such as Receptor-Interacting Serine/Threonine-Protein Kinase 1 (RIPK1) deficiency (100%), but was also common in immune-related actinopathies, including ARPC1B (66.7%) and dedicator of cytokinesis 8 (DOCK8, 14.3%) deficiencies, as well as in selected innate immunity defects, particularly CGD (23.1%) and CHAPLE disease (37.5%). The gene discussed is RIPK1; the disease is chronic granulomatous disease.