Recently, Giannini and colleagues demonstrated an up-regulation of inhibitory immune checkpoint mediators CD86 and CTLA-4, PD-L1/PD-L2 and PD-1, PVR and TIGIT, LAG3 and TIM3 and stimulatory immune checkpoint mediators GITR and GITR ligand, TNFRSF9, TNFRSF4, and TNFRSF4 ligand, and CD40, mainly in ATCs and at a lower extent in a subgroup of PTCs. The gene discussed is HAVCR2; the disease is Ehlers-Danlos syndrome, musculocontractural type.