The significantly upregulated activation of the inflammatory response IL1B in cells of severely affected PXE patients compared to both mildly affected patients and healthy controls, with respective fold changes of 8 and 51 (Supplementary Figure 5), may point toward a role for IL1B in the severity of the cardiovascular PXE phenotype though further experimental validation will be needed to make a more robust conclusion. Here, IL1B is linked to Pseudoxanthoma elasticum.