What is IPF?


Idiopathic pulmonary fibrosis (IPF)– is a general term for scarring (fibrosis) in the lung (pulmonary) and of an unknown cause (idiopathic). The term usually means that the scarring process is an active, ongoing process.

Idiopathic – This term is used for a disorder with no known cause.

Pulmonary – This is a general term for the lung.

Fibrosis – This term is used for scar or a scarring process.

Interstitial – Interstitial refers to the interstitium, which is the support structure of the lung. This structure is usually one to three cells thick. These cells give support to the blood vessels, lymphatic vessels, the small bronchiole airways, as well as the spherical oxygen exchange units called the alveoli. In IPF, the other interstitial pneumonias, and the interstitial lung diseases, there are too many cells in the interstitium. The interstitium becomes too thick for good exchange of oxygen.

Pneumonia – Pneumonia means that the alveoli (spherical oxygen exchange units) are filled with inflammation from an infection, other causes, or unknown cause.


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