Pulmonary fibrosis (PF)

Pulmonary fibrosis is a chronic, progressive, and often fatal respiratory condition characterized by the thickening and scarring of lung tissue, which progressively restricts oxygen transport into the bloodstream [1, 2]. From a pharmacological perspective, it is a complex disease indication rather than a single molecular target, involving dysregulated wound-healing responses across multiple cell types, including alveolar epithelial cells and myofibroblasts [3, 4]. Pathogenesis is driven by an interplay of various signaling molecules, most notably transforming growth factor-beta (TGF-beta), which promotes fibroblast-to-myofibroblast transition and excessive collagen deposition [5, 6]. Additionally, receptor tyrosine kinases such as those for platelet-derived growth factor (PDGF) and fibroblast growth factor (FGF) play critical roles in driving the proliferation of fibrotic cells [7]. Current FDA-approved therapies, nintedanib and pirfenidone, target these broad signaling networks to slow the decline of lung function, though they cannot reverse existing fibrosis [8, 9]. Emerging therapeutic strategies are focusing on more specific molecular targets, such as lysophosphatidic acid receptor 1 (LPA1) and integrin alpha-v beta-6, to provide more effective disease-modifying options [4, 10]. Clinical monitoring often relies on forced vital capacity (FVC) as a functional biomarker, alongside serum proteins like Krebs von den Lungen-6 (KL-6) and Matrix metalloproteinase-7 (MMP-7) for assessing disease activity and prognosis [11, 12].

Other names

Idiopathic pulmonary fibrosisIPFLung fibrosisInterstitial lung diseaseDiffuse parenchymal lung diseaseLung scarring

Inhibition of multiple receptor tyrosine kinases (including VEGFR, FGFR, and PDGFR) and modulation of transforming growth factor-beta (TGF-beta) signaling pathways.