Toll-like receptor 4 (TLR4) signaling through Myd88-dependent IRAK-M expression has been shown to be increased in peripheral blood cells from idiopathic pulmonary fibrosis patients compared to controls and was linked to the alternative macrophage activation, profibrotic phenotype, and collagen production [66,67]. This evidence concerns the gene TLR4 and idiopathic pulmonary fibrosis.