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  Oct 11, 2018

What is Idiopathic Pulmonary Fibrosis?

Idiopathic pulmonary fibrosis (IPF), also known as cryptogenic fibrosing alveolitis, is a health condition involving the progressive scarring and thickening of the lungs. This occurs without a known cause and can lead to the presentation of symptoms such as cough, shortness of breath, hypoxia and fatigue.


On a global basis, these are estimated to be 10.7 cases of IPF per 100,00 person-years for males and 7.4 cases for females. The prevalence of the condition is approximately 20 cases per 100,000 population for males and 13 cases for females.

There is a poor prognosis for patients with IPF, and the average survival following diagnosis is estimated to be between two and five years. The mortality rates range from 58 deaths per million people for women and 64 for men.


Although the cause of IPF is unknown, there are a number of factors that can contribute to the pathology and increase the probability that an individual will be affected by the condition. These include a history of smoking, an inherited genetic susceptibility and exposure to triggers in the environment, such as certain dust particles or fumes.

The normal structure and function of the lungs can change, slowly progressing with more damage. Initially, scar tissue forms on the alveoli in the lungs, which gradually gets thicker. Over time, this can interfere with the air passing through the lungs and can make it difficult to breathe and supply oxygen needed in the blood.

Most individuals that present with IPF are over the age of 60, and it is rare for the condition to be diagnosed before the age of 40. This is likely associated with the pathophysiology of the condition, especially the fact that continuous damage over time appears to be the prime risk factor.


Most patients that are diagnosed with idiopathic pulmonary fibrosis are over the age of sixty, and anyone over the age of 40 is considered to be at risk of the condition.