Pulmonary Fibrosis

IMPROVED LUNG FUNCTION

Pulmonary fibrosis (PF) is a progressive, interstitial lung disease characterized by scarring of the lung parenchyma that leads to irreversible loss of lung function. It is defined histopathologically by fibroblast proliferation, extracellular matrix deposition, and architectural distortion, with idiopathic pulmonary fibrosis (IPF) representing the most common and severe form. The disease arises from repeated alveolar epithelial injury, abnormal wound healing responses, fibroblast activation, and dysregulated immune signaling, leading to thickened, stiffened lungs with impaired gas exchange. Clinically, PF presents with dyspnea, chronic cough, reduced exercise tolerance, and low diffusion capacity. Progressive disease results in respiratory failure, pulmonary hypertension, and premature death, with median survival for IPF patients only 3–5 years after diagnosis.

Key Facts:

  • Epidemiology: Affects ~5 million people globally; IPF incidence is ~3–9 cases per 100,000 per year, with higher prevalence in older adults.
  • Unmet Need: No curative therapies; lung transplantation remains the only definitive treatment. Approved antifibrotic agents (nintedanib and pirfenidone) slow progression but do not halt or reverse fibrosis.
  • Economic Burden: Average cost of care for IPF patients can exceed $20,000 per year, with hospitalizations for acute exacerbations and lung transplantation driving substantial long-term expenses.
  • Clinical Opportunity: Zelpultide alfa is currently being developed as a novel therapeutic candidate to target and interrupt key disease pathways, with the goal of improving clinical outcomes and survival.
  • Investor Opportunity: With a growing aging population and limited effective treatments, the PF/IPF market is projected to expand significantly, with estimated multi-billion-dollar peak sales potential for innovative therapies.