Nevertheless, IL-1R1 blockade with anakinra has shown efficacy in mucosal healing in some pediatric patients with IL-10 receptor deficiency—a monogenic form of VEO-IBD marked by enhanced macrophage-mediated IL-1 production and an aggressive, Crohn’s-like phenotype with deep ulceration [172,173]. Here, IL1R1 is linked to inflammatory bowel disease.