Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrotic interstitial lung disease of unknown etiology characterized by progressive replacement of normal lung tissue with fibrotic scar tissue. This fibrosis impairs gas exchange, leading to progressive hypoxemia and dyspnea, primarily with exertion initially but progressing to dyspnea at rest. The disease demonstrates typical/usual interstitial pneumonia (UIP) pattern on imaging and histology. The pathophysiology involves aberrant wound healing responses following repeated epithelial injury, excessive fibroblast proliferation and activation, and excessive collagen deposition. Progressive pulmonary fibrosis leads to restrictive lung physiology, reduced lung capacity, and eventual respiratory failure. IPF has historically been associated with poor prognosis; however, antifibrotic drugs (pirfenidone, nintedanib) have demonstrated ability to slow progression and improve survival outcomes.