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What Is Pulmonary Fibrosis?

Pulmonary fibrosis is a disease where there is scarring of the lungs—called fibrosis—which makes it difficult to breathe. This is because the scarring causes the tissues in the lungs to get thick and stiff and makes it hard to absorb oxygen into the bloodstream.

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Pulmonary Fibrosis Types and Causes

Idiopathic Pulmonary Fibrosis (IPF)

The most common type of PF is IPF, which stands for idiopathic pulmonary fibrosis. This means this type of PF has no known cause. Approximately 50,000 new cases of IPF are diagnosed each year. Most IPF patients first start noticing symptoms between the ages of 50 and 70 years old. It is more common in men, but the number of cases of IPF in women is on the rise.

PF from Diseases

Some cases of PF are caused by autoimmune diseases like rheumatoid arthritis, scleroderma or Sjogren's syndrome. Certain viral infections and gastroesophageal reflux disease (GERD) are also risk factors for PF. GERD is a condition in which acid from your stomach backs up into your throat. Some people who have GERD may breathe in tiny drops of acid from their stomachs, which may injure the lungs.

PF from Exposures

PF can be caused by exposure to hazardous materials. Examples include occupational exposures such as asbestos or silica. Some cases of PF are caused by breathing in bird or animal droppings. Radiation treatments and certain types of medications can cause PF. Cigarette smoking also increases a person's risk of developing PF.

Familial PF

Familial PF is very rare. PF is considered familial when two or more members within the same family have idiopathic pulmonary fibrosis (IPF) or any other form of idiopathic interstitial pneumonia (IIP). There are genes that have been linked to PF but much is still unknown about this field. If someone in your family has had any type of interstitial lung disease, be sure to speak with your doctor and a genetic counselor about your risk.

Symptoms of 
Pulmonary Fibrosis

One reason pulmonary fibrosis often goes misdiagnosed is that symptoms of PF can be similar to symptoms of other lung diseases. Most people with PF develop symptoms between the ages of 50 and 70 years. If you have any of the symptoms below, talk with your doctor right away and be persistent if you feel your lung health is not improving. Also be sure to tell your doctor about any family history of lung disease or any exposure to risk factors you may have had. There are many types of lung disease that can cause pulmonary fibrosis. It is important that your doctor refers you to a specialist to make the right diagnosis.

Shortness of breath may mean that your body is not getting enough oxygen. Talk to your doctor right away and push for an accurate diagnosis.

  • Shortness of breath, particularly during exercise
  • Dry, hacking cough
  • Fast, shallow breathing
  • Gradual unintended weight loss
  • Tiredness
  • Aching joints and muscles
  • Clubbing (widening and rounding) of the tips of the fingers or toes

How Is Pulmonary Fibrosis Diagnosed?

Pulmonary fibrosis (PF) may be difficult to diagnose as the symptoms of PF are similar to other lung diseases. There are many different types of PF. If your doctor suspects you might have PF, it is important to see a specialist to confirm your diagnosis. This will help ensure you are treated for the exact disease you have.

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