Pulmonary Fibrosis

What is Pulmonary Fibrosis?

When your lung tissue develops damage and scar tissue, it can impact how your lungs function. This lung scarring, which can have a number of causes, results in Pulmonary Fibrosis and can impact your ability to breathe.

Lung scarring can be caused by tobacco use, environmental pollutants like metal dust and chemical gases, some medications such as those used in chemotherapy treatments for cancer, and some viral infections. If you have gastroesophageal reflux disease (GERD), where stomach acid backs up into your throat, you can get small drops of that acid in your lungs and incur damage.

Some people are genetically at risk for pulmonary fibrosis. Talk to your doctor if you have a close family member with the disease.

What are the Symptoms of Pulmonary Fibrosis?

If you have pulmonary fibrosis, you may find yourself struggling to take a full breath, especially when you exercise. Other symptoms include a dry cough, fast breathing to compensate for not being able to take a full breath, fatigue, aching muscles and unintended weight loss. Another tell-tale sign is clubbing, when the tips of your fingers and toes become rounder and wider.

Sometimes people with pulmonary fibrosis develop symptoms suddenly, but they usually develop slowly and worsen over time.

Pulmonary Fibrosis Causes

The exact cause of pulmonary fibrosis is unknown. It is known as an idiopathic disease, meaning that the exact cause is hard to identify or cannot be identified at all, despite intensive medical testing and analysis from medical professionals. Currently there are thought to be several potential causes for pulmonary fibrosis:

  • Autoimmune diseases: It is possible that some autoimmune diseases can cause pulmonary fibrosis. This happens when the immune system begins attacking the lungs and causes scarring.
  • Occupation and environmental exposure: High exposure to work related hazards, such as silica or coal dust, or general environmental hazards such as mold may contribute to pulmonary fibrosis.
  • Medicine: Certain medications used to treat conditions such as heart disease, seizures, some infections, and cancer have been linked to the development of pulmonary fibrosis.
  • Genetics: A small percentage of pulmonary fibrosis sufferers have a relative who also has the disease. This is known as familial pulmonary fibrosis. More research is being completed in this area, as a number of genes have been linked with the development of pulmonary fibrosis.

How is Pulmonary Fibrosis Treated?

There is no treatment that can cure pulmonary fibrosis, but some medications can lessen symptoms and prevent additional scar tissue from forming. Temporary use of corticosteroids can reduce inflammation, but the side effects prevent long-term use.

Oxygen therapy may be a good option if you aren’t getting enough oxygen in your blood. You can use supplemental oxygen to help you function in your day-to-day life.

Severe cases of pulmonary fibrosis may require a complete lung transplant.

Your doctor may also ask you to get vaccinated against the flu and pneumonia to lessen the chances of getting a respiratory disease that will worsen your condition.

Pulmonary Fibrosis Prevention

There are several risk factors associated with pulmonary fibrosis that might indicate one might develop the disease. Age is a large risk factor, with most people developing the disease after the age of 50. Another large possible predictor of the disease is having a family history of pulmonary fibrosis or having a genetic test that indicates genes linked with pulmonary fibrosis. In order to discover a possible connection, a genetic test and consultation with a genetic counselor is recommended if you think you might be at risk for pulmonary fibrosis.

There is no cure for the disease, and symptom onset can happen very quickly. The best method of prevention is to be aware of the genetic and age risk factors and to live a healthy lifestyle. Diet, exercise, and avoidance of cigarette smoking and excessive alcohol use may help, as these contribute to other diseases that might be associated with the development of pulmonary fibrosis. The medicines used to treat diseases such as heart disease and some cancers are known to be linked to pulmonary fibrosis. Protecting oneself if you are exposed to high levels of environmental and occupational pollutants is also necessary, as well as knowing the risks of working or living around certain pollutants.

Last Reviewed:
October 10, 2016
Last Updated:
January 17, 2018