IPF causes a change from a healthy lung (left) to one with scarring. This scarring may form a honeycomb pattern (right) later when looked at by HRCT or in lung tissue if a biopsy
is taken.

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How Your Lungs Work

It is important for you to learn how your lungs work so you can understand how idiopathic pulmonary fibrosis (IPF) affects them. Your lungs get oxygen from the air. The oxygen travels through the bloodstream to the heart. From there, the oxygen-rich blood is pumped to all the cells in the body.

How IPF affects the lungs

IPF causes scar tissue to form in the lungs. This scarring is permanent and irreversible. When this happens, the lungs become stiff and difficult to inflate. This causes lung function — also known as pulmonary function — to become restricted. Lungs scarred by IPF no longer work like healthy lungs. They cannot absorb as much oxygen and deliver oxygenated blood throughout the body.

Think of your lungs as a pair of sponges. When a sponge is wet, it easily absorbs water, spreads it around, and stays wet by using more water. Now, think of a sponge slowly drying out. Eventually it becomes too dry and does not work properly. This is a simple way for you to understand what happens when IPF scars the lungs.

How IPF Progresses

How quickly IPF worsens is different for each person. For some, IPF may worsen quickly. For others, IPF may not change for several years. But IPF eventually progresses in everyone.

The figure below shows 3 different ways in which IPF may progress.

Used with permission of the American Thoracic Society.
Copyright © 2016 American Thoracic Society.
Raghu et al. 2011. An official ATS/ERS/JRS/ALAT statement: idiopathic
pulmonary fibrosis: evidence-based guidelines for diagnosis and
management. Am J Respir Crit Care Med. 183(6):788-824.
The American Journal of Respiratory and Critical Care Medicine is an official journal of the
American Thoracic Society.

  • As is shown in line A, some people have “stable”
    disease in which case there is very little progression
  • The majority of patients with IPF experience a slow
    but steady worsening of their disease called "slow progression" (line B)
  • In the third group, IPF worsens fairly quickly. These
    people are said to have “rapid progression” (line D)

A small number of patients with IPF may experience a serious and sudden worsening of symptoms, known as an acute exacerbation ( ! ). These acute exacerbations cause a sudden and permanent worsening of IPF that leaves the patient with less lung function than they had before the attack (line C). Your doctor will determine if you’ve had an acute exacerbation.

Risk factors for IPF

The exact cause of IPF is not known. However, experts believe that certain factors may put people at greater risk. These include environmental factors, certain viral infections, some medicines, and whether other family members have IPF. However, IPF does not occur in all people with these factors.

Factors that may make you more susceptible to pulmonary fibrosis include:

Environmental factors:
  • Cigarette smoking
  • Some occupations such as farming, ranching, hair styling, stone cutting/polishing, metal working
Viral infections:
  • Epstein-Barr
  • Influenza A
  • Hepatitis C
  • HIV
  • Herpes 6
Genetic factors:
  • Sometimes IPF occurs in members of the same family
Lifestyle Changes

If you have IPF, your doctor may suggest that you make some lifestyle changes
such as:

  • Quit smoking. This is the most important thing you can do.
  • Speak with your doctor about activities that may be good for you such as pulmonary rehabilitation.
  • Eat a healthy diet. A full stomach can make it harder to breathe, so eat smaller, more frequent meals and drink plenty of water.
  • Get plenty of rest. Short naps during the day can make a big difference in how you feel.
  • Practice relaxation techniques. This can help you maintain a positive attitude. Many people find yoga and meditation to be helpful.
  • Join a support group. Talking with others can help you see how they have coped with the same issues.

IPF causes a change
from a healthy lung (left)
to one with scarring. This
scarring may form a
honeycomb pattern (right)
later when looked at by
HRCT or in lung tissue if
ia biopsy is taken.

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