We report for the first time that BLM-induced pulmonary fibrosis is associated with NF-κB activation (RelA), upregulation of fibrotic genes, enhanced inflammatory response and altered expression of bone morphogenic protein receptor A (BMPR1A) in WT mice; and all these alterations were prevented in IKBM mice treated with BLM. Here, NFKB1 is linked to pulmonary fibrosis.