Definition

What is pulmonary fibrosis?

Pulmonary fibrosis is a lung disease that occurs when lung tissue becomes damaged and scarred. This thickened, stiff tissue makes it more difficult for your lungs to work properly. As pulmonary fibrosis worsens, you become progressively more short of breath.

The scarring associated with pulmonary fibrosis can be caused by a multitude of factors. But in most cases, doctors can’t pinpoint what’s causing the problem. When a cause can’t be found, the condition is termed idiopathic pulmonary fibrosis.

The lung damage caused by pulmonary fibrosis can’t be repaired, but medications and therapies can sometimes help ease symptoms and improve quality of life. For some people, a lung transplant might be appropriate.

How common is pulmonary fibrosis?

Please discuss with your doctor for further information.

Symptoms

What are the symptoms of pulmonary fibrosis?

The common symptoms of pulmonary fibrosis are:

  • Shortness of breath (dyspnea)
  • A dry cough
  • Fatigue
  • Unexplained weight loss
  • Aching muscles and joints
  • Widening and rounding of the tips of the fingers or toes (clubbing)

The course of pulmonary fibrosis — and the severity of symptoms — can vary considerably from person to person. Some people become ill very quickly with severe disease. Others have moderate symptoms that worsen more slowly, over months or years.

Some people may experience a rapid worsening of their symptoms (acute exacerbation), such as severe shortness of breath, that may last for several days to weeks. People who have acute exacerbations may be placed on a mechanical ventilator. Doctors may also prescribe antibiotics, corticosteroid medications or other medications to treat an acute exacerbation.

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes pulmonary fibrosis?

In most cases, there is no known cause for the disease.

Things that may increase the risk of pulmonary fibrosis include:

  • Cigarette smoking
  • Certain viral infections
  • Exposure to environmental pollutants, including silica and hard metal dusts, bacteria and animal proteins, and gases and fumes
  • The use of certain medicines
  • Some families have at least two members who have pulmonary fibrosis.
  • Gastroesophageal reflux disease (GERD). GERD is a condition in which acid from your stomach backs up into your throat. Some people who have GERD may breathe in tiny drops of acid from their stomachs, which may injure the lungs.

Risk factors

What increases my risk for pulmonary fibrosis?

There are many risk factors for pulmonary fibrosis, such as:

  • Although pulmonary fibrosis has been diagnosed in children and infants, the disorder is much more likely to affect middle-aged and older adults.
  • Idiopathic pulmonary fibrosis is more likely to affect men than women.
  • Far more smokers and former smokers develop pulmonary fibrosis than do people who have never smoked. Pulmonary fibrosis can occur in patients with emphysema.
  • Certain occupations. You have an increased risk of developing pulmonary fibrosis if you work in mining, farming or construction or if you’re exposed to pollutants known to damage your lungs.
  • Cancer treatments. Having radiation treatments to your chest or using certain chemotherapy drugs can increase your risk of pulmonary fibrosis.
  • Genetic factors. Some types of pulmonary fibrosis run in families, and genetic factors may be a component.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is pulmonary fibrosis diagnosed?

There are many types of lung disease that can cause pulmonary fibrosis. It is important that your doctor refers you to a specialist to try and make the right diagnosis. Your doctor will do a physical exam. Your doctor may ask about your history of smoking, things in the air at your job that could irritate your lungs, your medication history, other medical conditions, your family’s medical history, and your hobbies.

Other tests for pulmonary fibrosis may include:

  • Chest X-ray
  • Breathing tests to find out how much lung damage you have
  • Blood tests to check for oxygen levels in your bloodstream, autoimmune disease, and for possible infections
  • High resolution CT scan of chest (HRCT)
  • Lung biopsy if diagnosis not able to be made by HRCT. The biopsy is done as a surgical procedure and removes small pieces of lung tissue to find a diagnosis.
  • Exercise testing to find out how well your lungs move oxygen and carbon dioxide in and out of your bloodstream.

How is pulmonary fibrosis treated?

The lung scarring that occurs in pulmonary fibrosis can’t be reversed, and no current treatment has proved effective in stopping progression of the disease. Some treatments may improve symptoms temporarily or slow the disease’s progression. Others may help improve quality of life. Doctors will evaluate the severity of your condition to determine the most appropriate treatment for your condition.

Medications

Your doctor may recommend newer medications, including pirfenidone (Esbriet) and nintedanib (Ofev). These medications may help slow the progression of idiopathic pulmonary fibrosis. Both medications have been approved by the Food and Drug Administration (FDA). Additional medications and new formulations of these medications are being developed but have not yet been FDA approved.

Nintedanib can cause side effects such as diarrhea and nausea. Side effects of pirfenidone include rash, nausea and diarrhea.

Researchers continue to study medications to treat pulmonary fibrosis.

Doctors may recommend anti-acid medications to treat gastroesophageal reflux disease (GERD), a digestive condition that commonly occurs in people with idiopathic pulmonary fibrosis.

Oxygen therapy

Using oxygen can’t stop lung damage, but it can:

  • Make breathing and exercise easier
  • Prevent or lessen complications from low blood oxygen levels
  • Reduce blood pressure in the right side of your heart
  • Improve your sleep and sense of well-being

You may receive oxygen when you sleep or exercise, although some people may use it all the time. Some people carry a canister of oxygen, making them more mobile.

Pulmonary rehabilitation

Pulmonary rehabilitation can help you manage your symptoms and improve your daily functioning. Pulmonary rehabilitation programs focus on:

  • Physical exercise to improve your endurance
  • Breathing techniques that may improve lung efficiency
  • Nutritional counseling
  • Counseling and support
  • Education about your condition

Lung transplant

Lung transplantation may be an option for people with pulmonary fibrosis. Having a lung transplant can improve your quality of life and allow you to live a longer life. However, a lung transplant can involve complications such as rejection and infection. Your doctor may discuss with you if a lung transplant may be appropriate for your condition.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage pulmonary fibrosis?

The following lifestyles and home remedies might help you cope with pulmonary fibrosis:

  • Stop smoking. If you have lung disease, it’s very important to stop smoking. Talk to your doctor about options for quitting, including smoking cessation programs, which use a variety of proven techniques to help people quit. And because secondhand smoke can be harmful to your lungs, avoid being around people who are smoking.
  • Eat well. People with lung disease may lose weight both because it’s uncomfortable to eat and because of the extra energy it takes to breathe. Yet a nutritionally rich diet that contains adequate calories is essential. Try to eat smaller meals more often during the day.Aim to eat a variety of fruits and vegetables, whole grains, low-fat or fat-free dairy products, and lean meats. Avoid trans fat and saturated fat, too much salt, and added sugars. A dietitian can give you further guidelines for healthy eating.
  • Get moving. Regular exercise can help you maintain your lung function and manage your stress. Aim to incorporate physical activity, such as walking or biking, into your daily routine. Talk to your doctor about which activities may be appropriate for you. If you require assistance with mobility over time, such as a wheelchair, look for activities or hobbies you can do that don’t require walking.
  • 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.
  • Get vaccinated. Respiratory infections can worsen symptoms of pulmonary fibrosis. Make sure you receive the pneumonia vaccine and an annual flu shot. It’s important that your family members also be vaccinated. Aim to avoid crowds during flu season.
  • Follow your treatment plan. You’ll need to have ongoing treatment from your doctor. Follow your doctor’s instructions, take your medications as prescribed, and adjust your diet and exercise as needed. Go to all of your doctor’s appointments.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Review Date: July 20, 2017 | Last Modified: July 20, 2017

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