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What is idiopathic pulmonary fibrosis (IPF)?

Lyall A. Gorenstein, MD
Thoracic Surgery (Cardiothoracic Vascular)
Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease characterized by chronic inflammation, accompanied by an uncontrolled healing response that causes progressive scarring or thickening (fibrosis) of tissues between the lung’s alveoli, or air sacs. The cause of IPF is unknown, although the body’s own immune response seems to play a major role. The exact incidence of IPF is unclear, but recent data suggest approximately 28 cases per 100,000 population. The usual age at diagnosis is between 40 and 70, but often there is X-ray evidence of disease two to five years before the diagnosis is made. The disease affects more men than women.

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