KIR3DL1 and systemic sclerosis: Further studies report that lack of KIR2DL2 inheritance increases SSc risk (Salim et al., 2010), and that coinheritance of canonical inhibitory receptor-ligand pair KIR3DL1-HLA-Bw4(The80) protects from disease (Mahmoudi et al., 2017), suggesting that activating KIR inheritance in the absence of compatible inhibitory interacting pairs may result in poorly restrained NK and T-cell activation and associated damaging inflammation.