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.
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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.