Thus, although the precise role in disease burden remains unclear, the pronounced immune cell recruitment (36, 37) and release via AIM2 of pro-inflammatory (IL-1α and IFN-α) and pro-fibrotic (TGF-β) mediators from circulating monocytes are likely to be involved in lung fibrotic-like changes in PC conditions. This evidence concerns the gene IFNA1 and pachyonychia congenita.