Novel drug delivery systems and disease models for pulmonary fibrosis.

Abstract

Pulmonary fibrosis (PF) is a serious and progressive lung disease which is possibly life-threatening. It causes lung scarring and affects lung functions including epithelial cell injury, massive recruitment of immune cells and abnormal accumulation of extracellular matrix (ECM). There is currently no cure for PF. Treatment for PF is aimed at slowing the course of the disease and relieving symptoms. Pirfenidone (PFD) and nintedanib (NDNB) are currently the only two FDA-approved oral medicines to slow down the progress of idiopathic pulmonary fibrosis, a specific type of PF. Novel drug delivery systems and therapies have been developed to improve the prognosis of the disease, as well as reduce or minimize the toxicities during drug treatment. The drug delivery routes for these therapies are various including oral, intravenous, nasal, inhalant, intratracheal and transdermal; although this is dependent on specific treatment mechanisms. In addition, researchers have also expanded current animal models that could not fully restore the clinicopathology, and developed a series of in vitro models such as organoids to study the pathogenesis and treatment of PF. This review describes recent advances on pathogenesis exploration, classifies and specifies the progress of drug delivery systems by their delivery routes, as well as an overview on the in vitro and in vivo models for PF research.