Progress in understanding and treating idiopathic pulmonary fibrosis: recent insights and emerging therapies

Abstract

Idiopathic pulmonary fibrosis (IPF) is a long-lasting, continuously advancing, and irrevocable interstitial lung disorder with an obscure origin and inadequately comprehended pathological mechanisms. Despite the intricate and uncharted causes and pathways of IPF, the scholarly consensus upholds that the transformation of fibroblasts into myofibroblasts-instigated by injury to the alveolar epithelial cells-and the disproportionate accumulation of extracellular matrix (ECM) components, such as collagen, are integral to IPF's progression. The introduction of two novel anti-fibrotic medications, pirfenidone and nintedanib, have exhibited efficacy in decelerating the ongoing degradation of lung function, lessening hospitalization risk, and postponing exacerbations among IPF patients. Nonetheless, these pharmacological interventions do not present a definitive solution to IPF, positioning lung transplantation as the solitary potential curative measure in contemporary medical practice. A host of innovative therapeutic strategies are presently under rigorous scrutiny. This comprehensive review encapsulates the recent advancements in IPF research, spanning from diagnosis and etiology to pathological mechanisms, and introduces a discussion on nascent therapeutic methodologies currently in the pipeline.

Keywords: etiology; idiopathic pulmonary fibrosis; pathogenesis; progress; treatment.

FIGURE 1

The pathogenesis of idiopathic pulmonary fibrosis. Alveolar epithelial cell injury induces increased TGF-β expression, which promotes ECM deposition and CTGF expression. Under the combined effect of CTGF and exosomes, fibroblasts proliferate and differentiate into myogenic cells. In addition, increased expression of MMPs and IGF-1 in IPF promotes EMT, which in turn affects myogenic cells. The increase in the number of myogenic cells further aggravates ECM deposition.

FIGURE 2

The mechanism of drugs for the treatment of IPF. The arrows in the figure represent the activating effect and the black lines represent the inhibiting effect. The different drugs mentioned above have different principles of action, but the ultimate goal of all drugs is to inhibit fibroblast production and reduce or even block the deposition of ECM.