Abstract Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease where excessive extracellular matrix (ECM) deposition and remodeling stiffens the lung, impeding its function. Many factors are known to contribute to the development of this fibrosis, but a lack of conclusive understanding endures because of their complex nature. The modification of ECM and the unique architecture of the lung are such factors in the propagation of IPF and not solely casualties.