In in a cohort of 286 patients with IL-10/IL-10R deficiency, gastrointestinal disorders occurred in all patients with perianal manifestations (92%), fistulae (60%), and colitis (50%) being the most prominent signs, Interestingly, perianal abscesses (57%) and complications beyond the gastrointestinal tract including failure to thrive (58%), susceptibility to infections (≤ 23%), lymphoproliferation (≤ 12%), dermatologic manifestations (49%), or rheumatologic disorders (13%) were strictly linked to IL-10R deficiences and did not occur in IL-10 deficient patients [114]. The gene discussed is IL10RA; the disease is colitis.