Pulmonary Fibrosis

Pulmonary fibrosis (PF) is a scarring and thickening in your lungs that makes it hard to breathe deeply. It’s a type of interstitial lung disease, which affects the tissue between the air sacs in your lungs (alveoli).

Hard, stiff lung tissues don’t expand as well as they should. If you have pulmonary fibrosis, you may get short of breath during daily tasks that never seemed tiring before. Pulmonary fibrosis is permanent and usually gets progressively worse over time (called progressive pulmonary fibrosis).

What is idiopathic pulmonary fibrosis?

Idiopathic pulmonary fibrosis is lung scarring without a known cause. Most cases of pulmonary fibrosis are idiopathic.

What are the symptoms of pulmonary fibrosis?

Pulmonary fibrosis symptoms include:

• Shortness of breath, especially during or soon after exertion
• Breathing in short, shallow spurts
• Dry cough that doesn’t go away
• Fatigue
• Unexplained weight loss
• Clubbed fingers
• Bluish, gray or white skin around your lips, eyes or nails (cyanosis)

What causes pulmonary fibrosis?

Experts think pulmonary fibrosis happens when your lungs don’t heal properly from damage or inflammation. Specific causes include:

• Connective tissue disease. These include rheumatoid arthritis (RA), lupus and scleroderma.
• Environmental exposures. Substances that can cause lung scarring include asbestos, silica and beryllium. Hypersensitivity pneumonitis — a type of allergy to certain molds, bacteria or bits of bird feathers or droppings — can also cause it.
• Granulomatous disease. These include sarcoidosis or Langerhans cell histiocytosis.
• Medications or treatments. These include amiodarone, nitrofurantoin, methotrexate and radiation therapy.
• Smoking.

In most cases, the specific cause is unknown.

What are the risk factors for pulmonary fibrosis?

In addition to the causes above, you might also be at a higher risk for pulmonary fibrosis if you:

• Are 65 or older
• Are male
• Have a family member with pulmonary fibrosis
• Have certain conditions caused by changes in your DNA, like dyskeratosis congenita (a rare type of bone marrow failure)

What are the complications of pulmonary fibrosis?

Scarred lung tissues have a hard time getting oxygen to the rest of your body and can make your heart work harder. This can lead to complications that include:

• Low oxygen in your blood (hypoxemia) and tissues (hypoxia)
• High blood pressure in your lungs (pulmonary hypertension)
• Collapsed lung
• Lung infections
• Respiratory failure
• Heart failure

How is pulmonary fibrosis diagnosed?

It might take a number of tests and imaging procedures to diagnose pulmonary fibrosis. Your provider will listen to your lungs and get pulmonary function tests to understand how your lungs are working. They’ll check for and rule out other conditions that affect your heart and lungs.

Tests and procedures

Specific tests and procedures providers might use to diagnose pulmonary fibrosis include:

• Chest X-ray
• High-resolution CT (HRCT) scan
• Pulmonary (lung) function tests
• Blood tests, including an arterial blood gas
• Bronchoscopy
• Biopsy

They may also use an echocardiogram to diagnose or rule out issues with your heart.

Can pulmonary fibrosis be cured?

No, there isn’t a cure for pulmonary fibrosis. Treatment focuses on easing symptoms, slowing down progression, and improving your quality of life.

Treatments for pulmonary fibrosis could include:

• Antifibrotic medications. Nintedanib (OFEV®) or pirfenidone (Esbriet®) may slow down lung scarring and preserve lung function.
• Corticosteroids. These are sometimes used to reduce inflammation.
• Oxygen therapy. Your provider will prescribe extra oxygen if you don’t have enough of it getting to your blood or tissues. It’s delivered through a mask or tube in your nose.
• Pulmonary rehabilitation. Breathing exercises and physical therapy can make your lungs stronger and breathing easier.
• Lung transplant. Some people with fibrosis are eligible for a lung transplant.
• Clinical trials. If you’re interested, your provider can help you join a research study of new medicines that can potentially help people.

You may also need medications to treat underlying causes (like autoimmune disease) or conditions that make scarring worse (like GERD).

How do I take care of myself?

If you have a PF diagnosis, it might be harder to fight off or recover from infections. Washing your hands frequently, disinfecting surfaces and avoiding crowded places — especially during cold and flu season, and when COVID-19 cases are high — might help you avoid getting sick frequently. Getting recommended vaccinations, like those for pneumonia, the flu, RSV, and COVID can also help minimize your risk.

When should I see my healthcare provider?

Talk to a healthcare provider if you have shortness of breath or get tired more easily with everyday tasks than you used to. Early diagnosis and treatment can help slow down the progression of lung disease.

If you have a connective tissue or granulomatous disease, talk to your healthcare provider about whether there are ways to reduce your risk of fibrosis.

When should I go to the ER?

Call 911 or go to the emergency room if you experience:

• Severe or sudden difficulty breathing
• Chest pain
• Confusion or altered mental state
• Bluish skin, lips or nails

What questions should I ask my doctor?

It might be helpful to ask your healthcare provider:

• What caused this scarring?
• What are my treatment options?
• How do I take my medication?
• When should I follow up with you?
• Am I eligible for lung transplantation?

Can pulmonary fibrosis be prevented?

Many causes of pulmonary fibrosis aren’t preventable. You can reduce your risk of lung scarring from environmental exposures by:

• Avoiding substances that can harm your lungs, such as asbestos, metal dust or chemicals, and wearing a respirator (a mask that filters particles from the air) if you have to work with them
• Avoiding things that can cause chronic allergic reactions, like hay, grain, bird droppings or feathers, and heating and cooling systems — and wearing a respirator mask if you have to work with them
• Not smoking or quitting smoking

What can I expect if I have pulmonary fibrosis?

Lung scarring is nearly always permanent (unless caused by a medication and caught early). If you have an underlying disease, management of it might help prevent further damage. If the cause is unknown, your healthcare provider will treat your symptoms and try to prevent more damage.

Healthcare providers can’t easily predict how pulmonary fibrosis will progress. Your symptoms may get worse very slowly, over years. In some cases, the disease may lead to severe symptoms quickly (over months).

How long can a person live with pulmonary fibrosis?

The life expectancy of someone with the most common form, idiopathic pulmonary fibrosis, is three to five years. But life expectancies for people with PF have been getting longer in recent years.

What are the stages of pulmonary fibrosis?

While there’s no official staging system for pulmonary fibrosis, some providers may describe pulmonary fibrosis as mild, moderate, severe or very severe. They base this diagnosis on your symptoms, lung function tests and imaging.

At what age does pulmonary fibrosis start?

Most cases of pulmonary fibrosis are diagnosed in people 65 and older.