Diagnosis
To diagnose pulmonary fibrosis, your doctor or other healthcare professional reviews your medical and family history and does a physical exam. You can talk about your symptoms and review any medicines you take. You also will likely be asked about any continuous or repeated contact with dusts, gases, chemicals or similar substances, especially through work.
During the physical exam, your healthcare professional listens carefully to your lungs while you breathe. Pulmonary fibrosis often occurs along with a crackling sound at the base of the lungs.
You may have one or more of these tests.
Imaging tests
- Chest X-ray. Images of the chest may show the scar tissue that is usually part of pulmonary fibrosis. Sometimes the chest X-ray may not show any changes. More tests may be needed to find out why you are short of breath.
- Computerized tomography (CT) scan. A CT scan combines X-ray images taken from many different angles to create images of structures inside the body. A high-resolution CT scan can be helpful in diagnosing pulmonary fibrosis and in finding out how much lung damage has occurred. Some kinds of fibrosis have certain patterns.
- Echocardiogram. An echocardiogram uses sound waves to look at the heart. The test can create pictures of the heart's structures. It also can create videos that show how the heart is working. This test can tell the amount of pressure in the arteries of the lungs and in the right side of the heart.
Lung function tests
Also called pulmonary function tests, these are done to find out how well your lungs are working:
- Spirometry. In this test, you breathe out quickly and forcefully through a tube connected to a machine. The machine measures how much air the lungs can hold and how quickly air moves in and out of the lungs.
- Lung volume test. This test measures the amount of air the lungs hold at different times when breathing in and out.
- Lung diffusion test. This test shows how well the body moves oxygen and carbon dioxide between the lungs and the blood.
- Pulse oximetry. This simple test uses a small device placed on one of the fingers to measure how much oxygen is in the blood. The percentage of oxygen in the blood is called oxygen saturation. Your healthcare professional may recommend a six-minute walking test with a check of your oxygen saturation.
- Exercise stress test. An exercise test on a treadmill or stationary bike may be used to monitor heart and lung function during activity.
- Arterial blood gas test. In this test, a sample of blood, usually taken from an artery in the wrist, is tested. The oxygen and carbon dioxide levels in the sample are measured.
In addition to showing whether you have pulmonary fibrosis, imaging and lung function tests can be used to check your condition over time and see how treatments are working.
Tissue sample
If other tests cannot find the cause of your condition, a small amount of lung tissue may need to be removed. This is called a biopsy. The biopsy sample is then examined in a laboratory to diagnose pulmonary fibrosis or rule out other conditions. One of these methods can be used to get a tissue sample:
Surgical biopsy. Although a surgical biopsy is invasive and has potential complications, it may be the only way to make the right diagnosis. This procedure may be done as a minimally invasive surgery called video-assisted thoracoscopic surgery (VATS). The biopsy also may be done as an open surgery called a thoracotomy.
During VATS, a surgeon inserts surgical instruments and a small camera through two or three small cuts between the ribs. The surgeon looks at the lungs on a video monitor while removing tissue samples from the lungs. During the surgery, a combination of medicines put you in a sleep-like state called general anesthesia.
During a thoracotomy, a surgeon removes a lung tissue sample through a cut that opens the chest between the ribs. This open surgery also is done using general anesthesia.
Bronchoscopy. In this procedure, very small tissue samples are removed — usually no larger than the head of a pin. A small, flexible tube called a bronchoscope is passed through the mouth or nose into the lungs to remove the samples. The tissue samples are sometimes too small to make the right diagnosis. But this form of biopsy also may be used to rule out other conditions.
Blood tests
You may have blood tests to look at your liver and kidney function. Blood tests also can check for and rule out other conditions.
More Information
Treatment
The lung scarring and thickening that occurs in pulmonary fibrosis cannot be repaired. And no current treatment has proved effective in stopping the disease from getting worse over time. Some treatments may improve symptoms for a time or slow how fast the disease worsens. Others may help improve quality of life.
Treatment depends on the cause of your pulmonary fibrosis. Doctors and other healthcare professionals evaluate how severe your condition is. Then together you can decide on the best treatment plan.
Medicines
If you have idiopathic pulmonary fibrosis, your healthcare professional may recommend the medicine pirfenidone (Esbriet) or nintedanib (Ofev). Both are approved by the U.S. Food and Drug Administration (FDA) for idiopathic pulmonary fibrosis. Nintedanib also is approved for other types of pulmonary fibrosis that get worse quickly. These medicines may help slow the worsening of pulmonary fibrosis and may prevent bouts when symptoms suddenly get worse.
Nintedanib can cause side effects such as diarrhea and nausea. Side effects of pirfenidone include nausea, loss of appetite and skin rash from sunlight. With either medicine, your healthcare professional uses regular blood tests to check how well the liver is working.
New medicines and therapies are being developed or tested in clinical trials but are not yet approved by the Food and Drug Administration (FDA). Researchers continue to study medicines to treat pulmonary fibrosis.
Doctors may recommend anti-acid medicines if you have symptoms of gastroesophageal reflux disease (GERD). GERD is a digestive condition that commonly occurs in people with idiopathic pulmonary fibrosis.
Oxygen therapy
Using extra oxygen, called supplemental oxygen, cannot stop lung damage, but it can:
- Make breathing and exercise easier.
- Prevent or lessen complications from low blood oxygen levels.
- Possibly lessen strain on the right side of the heart.
- Improve sleep and sense of well-being.
You may use oxygen when you sleep or exercise. But some people need oxygen all the time. Carrying a small tank of oxygen or using a portable oxygen concentrator can help you be more mobile.
Pulmonary rehabilitation
Pulmonary rehabilitation can help manage your symptoms and improve your ability to do daily tasks. Pulmonary rehabilitation programs focus on:
- Physical exercise to improve how much you can do.
- Breathing techniques that may improve how well your lungs use oxygen.
- Nutritional counseling.
- Emotional counseling and support.
- Education about your condition.
When symptoms suddenly get worse
When symptoms suddenly get worse, called an acute exacerbation, you may need more supplemental oxygen. In some cases, you may need mechanical ventilation in the hospital. In this treatment, a tube is guided into the lungs and attached to a machine that helps with breathing. Your healthcare professional may recommend antibiotics, corticosteroid medicines or other medicines when symptoms suddenly get worse.
Lung transplant
A lung transplant may be an option for some people with pulmonary fibrosis. Having a lung transplant can improve your quality of life and allow you to live a longer life. But a lung transplant can involve complications such as rejection and infection. After a lung transplant, you take medicines for the rest of your life. You and your healthcare team may discuss a lung transplant if it's thought to be the right treatment option for your condition.
Clinical trials
Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this condition.
Lifestyle and home remedies
Being actively involved in your treatment and staying as healthy as possible are essential to living with pulmonary fibrosis. It's important to:
- Stop smoking. If you have lung disease, it is important to stop smoking. Talk with your healthcare team about options for quitting, including smoking cessation programs. These use proven techniques to help people quit. Because secondhand smoke can be harmful to your lungs, avoid being around people who are smoking.
- Avoid other things that can irritate your lungs. Breathing indoor pollutants, such as fumes from heating fuel or chemicals, can irritate your lungs. So can outdoor pollutants, such as dust or car exhaust.
- Eat well. People with lung disease may lose weight both because eating is not comfortable and because of the extra energy it takes to breathe. A healthy diet that contains enough calories is needed. Try to eat smaller meals more often during the day. A dietitian can give you more information on healthy eating for your condition.
- Get moving. Regular exercise can help you keep your lung function and manage your stress. Aim to include physical activity, such as walking or biking, into your daily routine. Talk to your healthcare team about what activities may be best for you. If over time you need help getting around, such as using a wheelchair, look for active movements you can do that do not require walking. One example is tai chi.
- Take time to rest. Make sure to get enough rest. Taking time to rest can help you have more energy and cope with the stress of your condition. If you have problems sleeping, talk with your healthcare team.
- Get vaccinated. Respiratory infections, such as colds and flu, can worsen symptoms of pulmonary fibrosis. Make sure that you get the pneumonia vaccine, an annual flu shot and COVID-19 vaccines. It's important that your family members also be vaccinated. Try to stay out of crowds when possible.
- Follow your treatment plan. You usually need ongoing treatment from your healthcare team. Follow the care team's instructions. Take your medicines as prescribed. Adjust your diet and exercise as needed. Attend pulmonary rehabilitation sessions. Go to all of your appointments and contact your care team if symptoms worsen.
Coping and support
Pulmonary fibrosis is a life-long lung condition that worsens over time. Having pulmonary fibrosis can cause fear, depression and stress. Here are some tips that may help you cope.
- Learn about your condition. Understanding the condition and treatments can help you and your family cope.
- Spend time with family and friends. Let them know how they can support and help you.
- Talk to your doctor or other healthcare professional. Talk about your condition and how you feel. If you're depressed or anxious, your doctor may suggest that you see a mental health professional.
- Join a support group. Meeting with other people who have pulmonary fibrosis may help. You can talk to people who have had similar symptoms or treatments. You also can learn ways to cope.
- Learn about palliative care services. As your condition gets worse, your care team may suggest palliative care services. These services provide support for severe symptoms, such as providing relief from pain and helping to improve other symptoms. They also help you and your family discuss end-of-life issues and plan advance directives.
Preparing for your appointment
If your primary care doctor or other healthcare professional suspects a serious lung problem, you are likely to be referred to a pulmonologist. This is a doctor with training and skills in diagnosing and treating lung disorders.
Pulmonary fibrosis is a serious and complex disease. Take a friend or family member with you to your appointment. That person can take notes while you talk with your healthcare team, provide emotional support and help remember information that you may forget or miss.
What you can do
To get ready for your appointment, make a list of:
- Any symptoms you're having and for how long.
- Key medical information, including recent hospital stays and any medical conditions.
- Key personal information, including any type of work that increases your risk or any recent travel.
- All medicines, vitamins, herbs and other supplements that you take, including the doses.
- Questions to ask the healthcare professional.
Some basic questions to ask include:
- What is likely causing my symptoms?
- What kinds of tests do I need?
- What treatments do you recommend?
- I have other health conditions. How will pulmonary fibrosis affect them?
- Are there any things that I should do or not do?
Feel free to ask other questions during your appointment.
What to expect from your doctor
Some questions your healthcare professional is likely to ask include:
- What are your symptoms and when did they start?
- Are you getting treatment for any other medical conditions?
- What medicines and supplements have you taken in the past five years?
- Do you smoke? If so, how much and for how long?
- What type of work have you ever done, even if only for a few months?
- Do any members of your family have long-term lung disease of any kind?
- Have you ever had chemotherapy or radiation treatments for cancer?
- Do you have any other medical conditions?
Be ready to answer questions so that you have time to talk about what is most important to you.
Feb. 15, 2024