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The IPF Journey: Pathophysiology, Progression, and Patient Impact
Panelists discuss how idiopathic pulmonary fibrosis (IPF), a progressive and often fatal interstitial lung disease, presents significant clinical challenges due to its variable trajectory, limited treatment options, and poor prognosis—underscoring the importance of early diagnosis and specialized care and the need for therapies that can more effectively alter disease progression.
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease of unknown cause characterized by scarring (fibrosis) of the lung tissue, leading to a gradual decline in lung function. The pathophysiology involves repeated alveolar epithelial injury followed by abnormal wound healing, which promotes fibroblast proliferation and excessive extracellular matrix deposition. This fibrosis disrupts the normal lung architecture, impairing gas exchange and resulting in symptoms like chronic dry cough, fatigue, and progressive shortness of breath.
IPF primarily affects older adults, with most diagnoses occurring in individuals over the age of 60 years. It is more common in men than women and has been associated with a history of smoking and certain occupational exposures, although no direct cause has been identified. Genetic predisposition and environmental factors may also contribute. In the U.S., IPF is considered a rare disease but still affects approximately 100,000 people, with an estimated 30,000 to 40,000 new cases diagnosed annually.
The disease trajectory of IPF is highly variable, but it is generally progressive and associated with a poor prognosis. Median survival from diagnosis is around 3 to 5 years. Some patients experience a slow, steady decline in lung function, while others may have episodes of acute exacerbation that can lead to rapid deterioration. Treatment options are limited, primarily involving antifibrotic agents like nintedanib and pirfenidone, which can slow disease progression but do not reverse fibrosis. Lung transplantation remains the only curative option for eligible patients. Early diagnosis and referral to specialized care are critical to optimizing outcomes and managing the complex course of this debilitating disease.
