Pulmonary Fibrosis

What is it?

Pulmonary fibrosis is scarring of the lungs. In pulmonary fibrosis, scar tissue accumulates in the lungs after the lung tissue has been damaged. Pulmonary fibrosis makes it hard for oxygen to pass from your lungs into your blood. Pulmonary fibrosis may be linked to radiation therapy that has affected the lungs or certain chemotherapies. All patients getting radiation therapy that affects lung tissue will develop some degree of pulmonary fibrosis. The extent of the pulmonary fibrosis depends upon the type of treatment, the amount of tissue affected by the treatment, and the dose.

Pulmonary fibrosis can also be idiopathic (unknown cause), caused by an autoimmune disease, being around an irritant, a medication, or genetics. Radiation-induced pulmonary fibrosis often happens within 1-3 years after treatment. Fibrosis caused by chemotherapy often starts weeks to months after treatment.

The most common signs are cough and shortness of breath. Symptoms worsen as the amount of scar tissue increases. Other symptoms are:

• Fatigue.
• Weakness.
• Chest discomfort.
• Loss of appetite.
• Unexplained weight loss.

Your provider may order tests to see if you have pulmonary fibrosis and what is causing it. These tests can be:

• CT scan.
• Blood tests.
• Pulmonary function tests.
• Pulse oximetry.
• Bronchoscopy.
• Echocardiogram.
• Lung biopsy.

Management

Pulmonary fibrosis is managed by treating the symptoms:

• Rest as much as possible. Your provider may order bed rest if needed.
• Oxygen therapy, bronchodilators, and steroids may be given to help with breathing and oxygen levels.

When to contact your care team

You should contact your provider right away if you have symptoms of pulmonary fibrosis. Contact your care provider if you have any of the following:

• Trouble breathing.
• Shortness of breath.
• Blue-tinged lips, nails, or skin.