Description

Pulmonary fibrosis is a lung disease that occurs when lung tissue becomes damaged and scarred. This thickened, stiff tissue makes it more difficult for the lungs to function properly. The pulmonary fibrosis worsens with time. Some people may remain stable for a long time, but the condition worsens more quickly than in others. As it gets worse, people become more and more short of breath.

The scarring that occurs in the pulmonary fibrosis can be caused by many things. Often, doctors and other health professionals can't identify what is causing the problem. When the cause cannot be found, the condition is known as idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis usually occurs in middle-aged and older adults. Sometimes the pulmonary fibrosis is diagnosed in children and babies, but this is not common.

The lung damage caused by pulmonary fibrosis can't be repaired. Medications and therapies can sometimes help to slow down the rate of fibrosis, relieve symptoms, and improve quality of life. For some people, a lung transplant may be an option.

The pulmonary fibrosis care at the Mayo Clinic

Symptoms

The symptoms of pulmonary fibrosis can include:

  • Shortness of breath.
  • Dry cough.
  • Extreme tiredness.
  • The loss of weight is not the intention.
  • Pain in the muscles and joints.
  • The enlargement and rounding of the tips of the fingers or toes, it is called the night club.

How fast pulmonary fibrosis worsens with time and the severity of symptoms can vary greatly from person to person. Some people get sick very rapidly with severe disease. Others have mild symptoms that worsen more slowly, over months or years.

When symptoms suddenly get worse

In people with pulmonary fibrosis, especially idiopathic pulmonary fibrosis, shortness of breath may suddenly worsen for a couple of weeks or days. This is called an acute exacerbation. It can be deadly. The cause of an acute exacerbation may be another disease or condition, such as a lung infection. But usually the cause is not known.

When to see a doctor

If you have symptoms of pulmonary fibrosis, contact your doctor or other health care professional as soon as possible. If your symptoms get worse, especially if they get worse quickly, please contact with your health care team right away.

Causes

Pulmonary fibrosis is the scarring and thickening of the tissue around and between the air sacs called alveoli in the lungs. These changes make it more difficult for oxygen to pass into the bloodstream.

The damage to the lungs that results in pulmonary fibrosis can be caused by many different things. Examples include long-term exposure to certain toxins, radiation therapy, some medications, and certain medical conditions. In some cases, the cause of pulmonary fibrosis is not known.

His work and its surroundings

The type of work you do and where you work or live could be the cause or part of the cause of the pulmonary fibrosis. Having continued or repeated contact with toxins or pollutants (substances that can harm the quality of water, air or land — can damage the lungs, especially if you do not use the protective equipment. Examples include:

  • Silica dust.
  • The fibers of asbestos.
  • Hard metal powders.
  • Wood, coal and grain dusts.
  • The mold.
  • Bird and animal droppings.

Radiation treatments

Some people who receive radiation therapy to the chest, such as lung or breast cancer, signs of lung damage months or even years after treatment. How severe the damage is, you can depend on:

  • The amount of lung that was exposed to radiation.
  • The total amount of radiation given.
  • If chemotherapy is also used.
  • If there is underlying lung disease.

Medications

Many medications can cause damage to the lungs. Some examples include:

  • Chemotherapy. The drugs designed to kill cancer cells, such as methotrexate (Trexall, Otrexup, others), bleomycin and cyclophosphamide (Cytoxan), can damage the lung tissue.
  • Drugs for the heart. Some medicines used to treat irregular heartbeats such as amiodarone (Nexterone, Pacerone), can damage the lung tissue.
  • Some antibiotics. Antibiotics such as nitrofurantoin (Macrobid, Macrodantin) or ethambutol (Myambutol) may cause lung damage.
  • Anti-inflammatory medications. Certain anti-inflammatory medications such as rituximab (Rituxan) or sulfasalazine (Azulfidine) may cause lung damage.

Medical conditions

Lung damage can also be the result of a number of conditions, including:

  • Dermatomyositis, an inflammatory disease marked by muscle weakness and a skin rash.
  • Lupus, a disease that occurs when the body's immune system attacks its own tissues and organs.
  • Mixed connective tissue disease, which has a mix of symptoms of various disorders, such as lupus, scleroderma and polymyositis.
  • Pneumonia, an infection that inflames the air sacs in one or both lungs.
  • Polymyositis, an inflammatory disease that causes weakness in the muscles on both sides of the body.
  • Rheumatoid arthritis, an inflammatory disease that affects the joints and other body systems.
  • Sarcoidosis, an inflammatory disease that most often affects the lungs and lymph nodes.
  • Scleroderma, a group of rare diseases that involve the hardening and tightening of the skin, as well as problems in the interior of the body.

Idiopathic pulmonary fibrosis

Many substances and conditions can lead to pulmonary fibrosis. Even so, in many people, the cause is never found. But risk factors such as smoking or exposure to air pollution could be linked with the condition, even if the cause cannot be confirmed. The pulmonary fibrosis of unknown cause is called idiopathic pulmonary fibrosis.

Many people with idiopathic pulmonary fibrosis may also have gastroesophageal reflux disease, also called GERD. This condition occurs when the stomach acid backs up into the esophagus. GERD can be a risk factor for idiopathic pulmonary fibrosis or cause the condition to worsen more quickly. But more studies are needed.

Risk factors

Pulmonary fibrosis has been found in children and infants, but this is not common. Idiopathic pulmonary fibrosis is much more likely to affect middle-aged and older adults. Other types of pulmonary fibrosis, such as those caused by connective tissue disease, it can occur in younger people.

Factors that may increase the risk of pulmonary fibrosis include:

  • The habit of smoking. If you smoke now or used to smoke, you are at a higher risk of pulmonary fibrosis that people who have never smoked. People with emphysema are at higher risk, also.
  • Certain types of work. You have a higher risk of developing pulmonary fibrosis if you work in the mining, agriculture or construction. The risk is also higher if you have a continuous or repeated contact with pollutants can damage the lungs.
  • Cancer treatments. To have radiation treatments to the chest or the use of certain chemotherapy drugs can increase your risk of pulmonary fibrosis.
  • Genetics. Some types of lung fibrosis in families, so genes may play a role.

Complications

Complications of pulmonary fibrosis can include:

  • High blood pressure in the lungs. Called pulmonary hypertension, this type of high blood pressure that affects the arteries of the lungs. These are the pulmonary arteries. Stiffness and thickness of the arteries can slow or block the flow of blood through the lungs. This increases the pressure within the pulmonary arteries and in the lower right part of the heart of the camera, called the right ventricle.
  • Right-sided heart failure. This serious condition occurs when the heart is to the right of the camera has to pump harder than usual to move blood through the blocked portion of the pulmonary artery.
  • The respiratory failure. This is often the last stage of the long-term lung disease. It occurs when blood oxygen levels drop dangerously low.
  • The lung cancer. Long-standing pulmonary fibrosis, increases the risk of developing lung cancer.
  • Other lung problems. As pulmonary fibrosis worsens with time, it can lead to serious problems, such as blood clots in the lungs, a collapsed lung, or lung infections.

Diagnosis

To diagnose pulmonary fibrosis, or your doctor or other health care professional to review your medical and family history and do a physical exam. You can talk about your symptoms and review of medicines that you take. It is also likely that you will be asked about any continuous or repeated contact with dusts, gases, chemicals or similar substances, especially in the work.

During the physical exam, your health care professional will listen to your lungs while you breathe. The pulmonary fibrosis often occurs along with a crackling sound in the base of the lungs.

You can have one or more of these exams.

Imaging tests

  • X-ray of the chest. Imaging of the chest can show you the scar tissue that normally forms part of the pulmonary fibrosis. Sometimes a chest x-ray may not show any change. More tests may be needed to find out why you're short of breath.
  • Computed tomography (CT scan). A ct scan combines X-ray images taken from different angles to create images of the internal structures of the body. A high-resolution CT scanning may be useful in the diagnosis of pulmonary fibrosis and in the search of how much lung damage has occurred. Some types of fibrosis have certain patterns.
  • The echocardiogram. An echocardiogram uses sound waves to examine the heart. The test can create images of the structures of the heart. You can also create videos that show how the heart is working. This test can determine the amount of pressure in the arteries of the lungs and on the right side of the heart.

Pulmonary function tests

Also called pulmonary function test, this is done to find out how well your lungs are working:

  • Spirometry. In this test, you breathe quickly and with force through a tube connected to a machine. The machine measures the amount of air that the lungs can hold and how fast air moves in and out of the lungs.
  • The volume of the test lung. 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 the amount of oxygen in the blood. The percentage of oxygen in the blood is called the oxygen saturation. Your health care professional may recommend a six-minute walk test with a check of oxygen saturation.
  • Exercise stress test. An exercise test on a treadmill or stationary bike can be used to control the heart and lung function during the activity.
  • Arterial Blood Gas Analysis. In this test, a sample of blood, usually taken from an artery in the wrist, is the proof. The oxygen and carbon dioxide levels in the sample is measured.

In addition to a show if you have pulmonary fibrosis, image, and lung function tests can be used to check your condition over time and see how the treatments are working.

Tissue sample

If other tests can't 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 examined in a laboratory to diagnose pulmonary fibrosis or rule out other conditions. One of these methods can be used to obtain a sample of tissue:

  • Surgical biopsy.Although a surgical biopsy is invasive and has the potential of complications, you may be the only way to make the correct diagnosis. This procedure can be done as a minimally invasive surgery called video-assisted thoracoscopic surgery (VATS). The biopsy may also be done as an open surgery called a thoracotomy. DuringVATS, a surgeon inserts surgical instruments and a small camera through two or three small incisions between the ribs. The surgeon looks at the lungs on a video monitor, while the removal of tissue samples from the lungs. During the surgery, a combination of medications that put you in a sleep-like state called general anesthesia. During a thoracotomy, a surgeon removes a sample of lung tissue through a cut that opens the chest between the ribs. This open surgery also makes use of general anesthesia. The bronchoscopy.In this procedure, a very small tissue samples are removed (usually not 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 often too small to make the correct diagnosis. But this form of biopsy can also be used to rule out other conditions.
  • The bronchoscopy.In this procedure, a very small tissue samples are removed (usually not 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 often too small to make the correct diagnosis. But this form of biopsy can also be used to rule out other conditions.

Surgical biopsy. Although a surgical biopsy is invasive and has the potential of complications, you may be the only way to make the correct diagnosis. This procedure can be done as a minimally invasive surgery called video-assisted thoracoscopic surgery (VATS). The biopsy may also 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 incisions between the ribs. The surgeon looks at the lungs on a video monitor, while the removal of tissue samples from the lungs. During the surgery, a combination of medications that put you in a sleep-like state called general anesthesia.

During a thoracotomy, a surgeon removes a sample of lung tissue through a cut that opens the chest between the ribs. This open surgery also makes use of general anesthesia.

The bronchoscopy. In this procedure, a very small tissue samples are removed (usually not 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 often too small to make the correct diagnosis. But this form of biopsy can also be used to rule out other conditions.

Blood tests

You may have blood tests to check your liver and kidney function. Blood tests can also check for and rule out other conditions.

Treatment

The lung scarring and thickening that occurs in the pulmonary fibrosis can't be repaired. And no treatment has been shown to be effective in stopping the disease from getting worse with time. Some treatments may improve symptoms for a while or slow the rapid worsening of the disease. Others can help to improve the quality of life.

The treatment depends on the cause of the pulmonary fibrosis. Doctors and other health care professionals to assess the severity of the disease. Then, you can decide on the best treatment plan.

Medications

If you have idiopathic pulmonary fibrosis, your health care professional may recommend medicine pirfenidone (Esbriet) or nintedanib (Ofev). Both are approved by the Food and Drug Administration (FDA) for idiopathic pulmonary fibrosis. Nintedanib is also approved for other types of pulmonary fibrosis, which rapidly get worse. These medications can help to slow the worsening of pulmonary fibrosis and may prevent the attacks when symptoms suddenly get worse.

Nintedanib may cause side effects such as diarrhea and nausea. Side effects of pirfenidone include nausea, loss of appetite, and a rash on the skin from sunlight. With either medication, your health care professional uses blood tests to see how well the liver is working.

New drugs and therapies that are being developed or tested in clinical trials, but are not yet approved by the Food and Drug Administration (FDA). Researchers continue to study the drug for the treatment of pulmonary fibrosis.

Doctors may recommend anti-acid medications if you have symptoms of gastroesophageal reflux disease (GERD). GERD is a digestive condition that occurs commonly in people with idiopathic pulmonary fibrosis.

Oxygen therapy

The use of supplemental oxygen, called supplemental oxygen, you can't stop damage to the lungs, but it can:

  • Do the breathing and the easiest exercise.
  • To prevent or reduce the complications of low levels of oxygen in the blood.
  • Decrease the tension on the right side of the heart.
  • To improve sleep and feeling of well-being.

You can use oxygen when you sleep, or exercise. But some people need oxygen all the time. The realization of a small tank of oxygen or the use of a portable oxygen concentrator can help you to be more mobile.

Pulmonary rehabilitation

Pulmonary rehabilitation can help manage your symptoms and improve your ability to do everyday tasks. Pulmonary rehabilitation programs focus on:

  • Physical exercise to improve the way in which you can do a lot.
  • The breathing techniques that can improve how well your lungs to use oxygen.
  • Nutritional advice.
  • Emotional support and advice.
  • Education about your condition.

When symptoms suddenly get worse

When symptoms suddenly get worse, she 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 that is inserted into the lungs and connected to a machine that helps you breathe. Your health care professional may recommend antibiotics, corticosteroid medicines or other medications 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 may involve complications such as rejection and infection. After a lung transplant, taking medications for the rest of your life. You and your health care team can talk about a lung transplant if it's thought to be the treatment option for your condition.

Lifestyle and home remedies

Be actively involved in your treatment and stay as healthy as possible are essential for living with pulmonary fibrosis. It is important to:

  • Stop smoking. If you have lung disease, it is important to quit smoking. Talk with your health care team about the options to quit smoking, including smoking cessation programs. These used techniques proven to help people quit smoking. Due to that the second-hand smoke can be harmful to the lungs, to avoid being around people who smoke.
  • Avoid other things that can irritate the lungs. The breath of indoor pollutants, such as vapors of heating fuel or chemicals can irritate the lungs. So you can make the air free of contaminants, such as dust and car exhaust.
  • Eat well. People with lung disease may lose weight because eating is not comfortable and due to the extra energy you need to breathe. A healthy diet that contains enough calories you will need. Try eating smaller meals more often during the day. A dietitian can give you more information about the healthy diet for your condition.
  • Get moving. Regular exercise can help to maintain lung function and handle the stress. Aim to include physical activity, such as walking or bicycling, into your daily routine. Talk with your health care team about what activities may be best for you. If, during the time that you need help to get around, such as the use of a wheelchair, looking for active movements that you can do that do not require walking. An example is tai chi.
  • Take time to rest. Make sure you get enough rest. Take time to relax can help you have more energy and to deal with the stress of their condition. If you have trouble sleeping, talk with your health care team.
  • Get vaccinated. Respiratory tract infections such as colds and the flu, can worsen the symptoms of pulmonary fibrosis. Make sure that you receive the pneumonia vaccine, an annual vaccination against the flu and COVID-19 vaccines. It is important that the members of your family should also be vaccinated. Try to stay out of the crowds when possible.
  • Follow your treatment plan. You usually need continuous treatment of your health care team. Follow the care instructions on the computer. Take your medicines as prescribed. Adjust your diet and exercise as needed. Attend sessions of pulmonary rehabilitation. Go to all your appointments and contact with your health care team if the symptoms get worse.

Coping and support

Pulmonary fibrosis is a life-long lung condition that gets worse over time. Have pulmonary fibrosis can cause fear, depression, and stress. Here are some tips that can help you cope.

  • Learn about your condition. The understanding of the disease and its treatments can help you and your family cope.
  • Spending time with family and friends. Let them know how they can support and help.
  • Talk with your doctor or other health care 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 can help. You can talk with people who have had similar symptoms or treatments. You can also learn ways to cope.
  • Learn about the palliative care services. As your condition gets worse, your health care team can suggest palliative care services. These services provide support for severe symptoms, such as pain relief and help improve other symptoms. They also help you and your family to discuss end of life issues, and plan directives.

Preparing for your appointment

If your primary care doctor or other health care professional suspects a serious lung problem, it is likely that you will be referred to a pulmonologist. This is a doctor with special training and skill in the diagnosis and treatment of lung disorders.

Pulmonary fibrosis is a serious and complex disease. Bring a friend or family member with you to your appointment. This person can take notes as you talk with your health care team, provide emotional support and help to remember the information that you may forget or be lost.

What you can do

To prepare for your appointment, make a list of:

  • Any of the symptoms that you have and for how long.
  • Key medical information, including the recent hospital stays, and any medical condition.
  • Key personal information, including any type of work that increase your risk or of any recent travel.
  • All the drugs, vitamins, herbs and other supplements that you are taking, including the dosage.
  • Questions to ask the health professionals.

Some basic questions to ask include:

  • What is likely causing my symptoms?
  • What kinds of tests do I need?
  • What treatments are recommended?
  • I have other health conditions. How is pulmonary fibrosis affects them?
  • There are things that I should do or not do?

Feel free to ask questions during your appointment.

What to expect from your doctor

Some of the questions to your health professional is likely to ask include:

  • What are the symptoms and when do they start?
  • Are you being treated for any medical condition?
  • What medications and supplements that have been adopted in the past five years?
  • Do you smoke? If so, how much and for how long?
  • What type of work you have done, if only for a couple of months?
  • Do any of the members of your family have a long-term lung disease of any kind?
  • Have you ever had chemotherapy or radiation treatments for cancer?
  • Do you have any other medical condition?

Be prepared to answer questions that you have time to talk about what is most important to you.

Symptoms and treatment of Pulmonary fibrosis