Idiopathic Pulmonary Fibrosis
Small Molecule Therapeutics
Pulmonary fibrosis (PF) is a chronic and progressive interstitial lung condition characterized by excessive extracellular matrix and organization deposition. Idiopathic pulmonary fibrosis (IPF) is the most common form of PF and affects approximately 3 million people worldwide. IPF is a chronic and usually fatal lung disease in which the lungs become scarred, and breathing becomes progressively more difficult. Unfortunately, the prognosis of IPF patients remains poor, with a median survival of 2 to 3 years after diagnosis, mainly due to a lack of effective treatment options; this is worse than some cancers.
Currently, two treatments are approved to slow disease progression. Neither drug benefits IPF patients, and lung transplants often become the last resort.
Recent literature suggests a crucial role for lung resident mesenchymal stem cells (LR-MSCs) via a fibroblastic trans-differentiation event in idiopathic pulmonary fibrosis (IPF) pathogenesis. Aberrant activation of Wnt/β-catenin signaling occurs in all fibrotic lung diseases and is relevant to differentiating mesenchymal stem cells (MSCs). The Wnt/β-catenin signaling is activated in LR-MSCs and could be a potential target for novel treatment approaches. The Wnt/β-catenin suppression could modify disease processes by slowing or preventing fibrosis in IPF.