What is Pulmonary Fibrosis

Pulmonary fibrosis is a type of lung disease that causes scarring. Over time, the scar tissue builds up in the air sacs of your lungs (alveoli). This makes it hard for you to breathe. Less oxygen can get into your blood.

Scarring from pulmonary fibrosis gets worse over time. This damage is permanent and may lead to other serious health problems.

What are the causes?

There are many different causes of pulmonary fibrosis. Sometimes the cause is not known. This is called idiopathic pulmonary fibrosis. Other causes include:

• Exposure to chemicals and substances found in agricultural, farm, construction, or factory work. These include mold, asbestos, silica, metal dusts, and toxic fumes.
• Sarcoidosis. In this disease, areas of inflammatory cells (granulomas) form and most often affect the lungs.
• Autoimmune diseases. These include diseases such as rheumatoid arthritis, systemic sclerosis, or connective tissue disease.
• Taking certain medicines. These include drugs used in radiation therapy or used to treat seizures, heart problems, and some infections.

What increases the risk?

You are more likely to develop this condition if:

• You have a family history of the disease.
• You are older. The condition is more common in older adults.
• You have a history of smoking.
• You have a job that exposes you to certain chemicals.
• You have gastroesophageal reflux disease (GERD).

What are the signs or symptoms?

Symptoms of this condition include:

• Difficulty breathing that gets worse with activity.
• Shortness of breath (dyspnea).
• Dry, hacking cough.
• Rapid, shallow breathing during exercise or while at rest.
• Bluish skin and lips.
• Loss of appetite.
• Weakness.
• Weight loss and fatigue.
• Rounded and enlarged fingertips (clubbing).

How is this diagnosed?

This condition may be diagnosed based on:

• Your symptoms and medical history.
• A physical exam.

You may also have tests, including:

• A test that involves looking inside your lungs with an instrument (bronchoscopy).
• Imaging studies of your lungs and heart.
• Tests to measure how well you are breathing (pulmonary function tests).
• Blood tests.
• Tests to see how well your lungs work while you are walking (pulmonary stress test).
• A procedure to remove a lung tissue sample to look at it under a microscope (biopsy).

How is this treated?

There is no cure for pulmonary fibrosis. Treatment focuses on managing symptoms and preventing scarring from getting worse. This may include:

• Medicines, such as:
  • Steroids to prevent permanent lung changes.
  • Medicines to suppress your body’s defense system (immune system).
  • Medicines to help with lung function by reducing inflammation or scarring.
• Ongoing monitoring with X-rays and lab work.
• Oxygen therapy.
• Pulmonary rehabilitation.
• Surgery. In some cases, a lung transplant is possible.

Follow these instructions at home:

Medicines

• Take over-the-counter and prescription medicines only as told by your health care provider.
• Keep your vaccinations up to date as recommended by your health care provider.

General instructions

• Do not use any products that contain nicotine or tobacco, such as cigarettes and e-cigarettes. If you need help quitting, ask your health care provider.
• Get regular exercise, but do not overexert yourself. Ask your health care provider to suggest some activities that are safe for you to do.
  • If you have physical limitations, you may get exercise by walking, using a stationary bike, or doing chair exercises.
  • Ask your health care provider about using oxygen while exercising.
• If you are exposed to chemicals and substances at work, make sure that you wear a mask or respirator at all times.
• Join a pulmonary rehabilitation program or a support group for people with pulmonary fibrosis.
• Eat small meals often so you do not get too full. Overeating can make breathing trouble worse.
• Maintain a healthy weight. Lose weight if you need to.
• Do breathing exercises as directed by your health care provider.
• Keep all follow-up visits as told by your health care provider. This is important.

Contact a health care provider if you:

• Have symptoms that do not get better with medicines.
• Are not able to be as active as usual.
• Have trouble taking a deep breath.
• Have a fever or chills.
• Have blue lips or skin.
• Have clubbing of your fingers.

Get help right away if you:

• Have a sudden worsening of your symptoms.
• Have chest pain.
• Cough up mucus that is dark in color.
• Have a lot of headaches.
• Get very confused or sleepy.

Summary

• Pulmonary fibrosis is a type of lung disease that causes scar tissue to build up in the air sacs of your lungs (alveoli) over time. Less oxygen can get into your blood. This makes it hard for you to breathe.
• Scarring from pulmonary fibrosis gets worse over time. This damage is permanent and may lead to other serious health problems.
• You are more likely to develop this condition if you have a family history of the condition or a job that exposes you to certain chemicals.
• There is no cure for pulmonary fibrosis. Treatment focuses on managing symptoms and preventing scarring from getting worse.