In the IEI/MBLdef group, 11 patients were diagnosed with CVID, two patients with XLA, one patient with WHIM syndrome, one with an unreported deleterious mutation in the ifngr1 gene (NM_000416.2:c.110T>C) as well as three patients with autoinflammatory disorders (two patients with CAPS, one with Muckle-Wells syndrome). This evidence concerns the gene IFNGR1 and common variable immunodeficiency.