Reduced IL-1R8 expression has been described in patients affected by psoriatic arthritis (Batliwalla et al., 2005) while SIGIRR variants (characterized by defective SIGIRR function) have been found in humans in association with necrotizing enterocolitis (Sampath et al., 2015) and with systemic lupus erythematosus (SLE; [Zhu et al., 2014]), all pathologies being characterized by cognitive defects and neurodevelopmental impairment (Husted et al., 2013; Rees et al., 2007; Calderón et al., 2014; Muscal et al., 2010). The gene discussed is IL1RAPL1; the disease is systemic lupus erythematosus.