What is IPF?

Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease that causes permanent damage to the lungs.


Tissue damage caused by IPF may be due to abnormal healing that occurs in response to minor injuries in the deepest tissues inside the lungs. As a result of these injuries, air cells (alveoli) in the lungs thicken and harden, causing scarring (fibrosis). This makes it difficult for the oxygen you inhale to enter the bloodstream.


  • Although IPF is a rare disease, it is expected to be seen in 3 million people in the world.
  • The disease is more common in men.
  • Most IPF patients are over the age of 50..

In the early stage of the disease, normal tissue is adjacent to scarred tissue. As the disease progresses, fibrosis spreads. Over time, progressing fibrosis reduces the lungs' ability to transport oxygen into the bloodstream. There is currently no treatment available to repair the damage caused by IPF.



IPF: A disease with unknown reasons

Being diagnosed with IPF means that, unlike other forms of interstitial lung disease, the cause of lung scarring has not been identified. The medical community has not yet determined why IPF develops in some people, but not in others with similar factors.

Although the genetic factors in this disease are not well understood, scientists believe that in some cases IPF may be due to the interaction between the environment and the person's genetic (hereditary) potential for the disease.



Potential risk factors

There are no causes proven to lead to IPF. However, certain factors such as smoking may increase the risk of developing IPF.

Smoking: There is a strong relationship between the history of smoking and IPF, especially in people who have smoked a lot or smoked for many years. However, there are also patients with IPF who have never smoked.

Gastroesophageal reflux disease: Some scientists think that this digestive system disorder can cause repeated minor injuries to the lung tissue.

Infection: The relationship between infection and IPF has been examined in numerous studies; however, the findings are not conclusive.

Environmental pollutants: Exposure to airborne particles from metal and wood, farming, bird breeding, stone cutting/polishing, livestock or farm dust may increase the risk of IPF.


Age is a risk factor.

IPF typically affects individuals aged 50 and over. However, it can also be diagnosed in early adulthood.

What are the indications and symptoms of IPF?

Most people learn that they have IPF only after experiencing symptoms or after the disease progresses. In some cases, patients learn that they have IPF, after the determination of IPF-related lung scarring pattern in tests intended for other lung problems.


Early indications and symptoms of IPF may include:

  • Nonproductive persistent cough
  • Shortness of breath, sometimes visible even during regular daily activities
  • The doctor hears Velcro crackles in the base of the lungs


Over time, symptoms may intensify or more symptoms may appear:

  • You may experience shortness of breath even when you are resting.
  • Your dry cough may become drier and you may experience cough attacks that you cannot control.
  • You may easily become fatigued even during light activities.
  • Your fingers may develop clubbing in time.
  • You may need to take oxygen supplement therapy because of decreased oxygen saturation in your blood.


Progression of the disease may differ from person to person. In some people, symptoms caused by lung damage due to IPF start rapidly. Others may experience slower progress. It is difficult to fully predict the progression of the disease; therefore, doctors are not sure how fast IPF will progress in a patient.


Many factors can affect your prognosis, such as other health problems or advanced age. On average, most patients live between 2 and 5 years after being diagnosed. Some people with IPF live for 10 years or more after being diagnosed, while others' health rapidly deteriorates.


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