Pulmonary Fibrosis
Last Updated 05/08/2020
Authors:Marie M. Budev, DO, FCCP; Samantha D’Annunzio, MD; and Sheetal Gandotra, MD
About Pulmonary Fibrosis
Pulmonary fibrosis is a serious lung disease that causes the lung tissue to become scarred and stiff.
- In most cases, doctors don’t know what causes pulmonary fibrosis. When doctors cannot identify the cause, the disease is called idiopathic pulmonary fibrosis (IPF).
- IPF is a type of interstitial lung disease (ILD), which means that it affects the interstitium, or the tissue around the air sacs of the lung.
- There is no cure for IPF
How Pulmonary Fibrosis affects your body
Normal lungs have no scarring, so oxygen can pass easily from the environment into the air sacs of the lungs. When air reaches the air sacs, it is absorbed into the bloodstream so that your blood can receive oxygen. In pulmonary fibrosis, the lungs are stiff because of scarring, called fibrosis. This fibrosis makes it harder for oxygen to pass through the air sacs in the lung, which in turn reduces the amount of oxygen that can passes into the bloodstream.
Fibrosis stiffens the lungs, reducing their size and capacity. This decrease in lung ability can be seen on breathing tests, also known as lung function tests. These tests may be used to diagnose or monitor pulmonary fibrosis over time. As the fibrosis progresses, it may become harder to breathe, and you may need oxygen to help you breathe.
The scarring that happens with pulmonary fibrosis cannot be reversed or repaired. Unfortunately, there is no known cure for pulmonary fibrosis. Unlike other common long-term diseases such as diabetes or high blood pressure, pulmonary fibrosis is rare.
Pulmonary fibrosis affects up to an estimated 132,000 people in the United States. The symptoms of pulmonary fibrosis are similar to those of other lung diseases, including asthma and chronic obstructive lung disease. A correct diagnosis of pulmonary fibrosis is important so that a doctor may be able to help treat symptoms like shortness of breath.
- Pulmonary fibrosis can occur at any age but usually happens between the ages 50 and 70.
- Pulmonary fibrosis occurs more often in men than women.
- Pulmonary fibrosis occurs to people of all races, ethnicities, and social statuses.
What causes Pulmonary Fibrosis?
In most cases of pulmonary fibrosis, the cause isn’t known. In other cases, the causes of pulmonary fibrosis may include:
- Being exposed to silica, asbestos, or grain dust
- Environmental factors, including bird and animal droppings
- Radiation treatments for cancer
- Medications, including chemotherapy drugs, some heart medications, and some antibiotics
- Other medical illnesses
Living Well
Patient Guide
Pulmonary Fibrosis
Infographic
Pulmonary Fibrosis
Myths Busted
The Living Well with Pulmonary Fibrosis patient guide will introduce you to pulmonary fibrosis – its causes, symptoms and risk factors; how it is diagnosed; and how it is treated; and how to manage the condition to minimize its impact.
This patient education material was partially funded by the Feldman Family – Mitch Feldman, Laury Feldman and Mara Fox.
Symptoms of Pulmonary Fibrosis
How severe symptoms are varies from person to person. Some people will get very sick quickly when the disease is severe. Other people will have mild symptoms for years and the disease will remain mild.
How pulmonary fibrosis develops also varies from person to person, and the overall course of the disease can be unpredictable. The disease can change quickly, even for people who have been stable for years.
Signs and symptoms of pulmonary fibrosis include:
- Shortness of breath
- A dry cough
- Fatigue
- Weight loss
- Muscle and joint aching
What are the risk factors for Pulmonary Fibrosis?
- Age. Pulmonary fibrosis is more likely in those aged 50 to 70 years than in younger people.
- Smoking. More smokers or former smokers develop pulmonary fibrosis than nonsmokers.
- Occupation. Certain occupations, including mining, farming, and construction, may increase the risk for pulmonary fibrosis.
- Cancer treatment. Radiation treatments and certain chemotherapy medications can increase the risk for pulmonary fibrosis.
- Genetic factors. Some types of pulmonary fibrosis may occur more often in families because of a genetic factor.
Diagnosing Pulmonary Fibrosis
Your health care provider will give you a physical examination that includes listening to your lungs for abnormal breath sounds. Your provider may also perform tests to determine whether you have pulmonary fibrosis and how it has affected your body. These tests may include blood tests, imaging tests, and lung function tests. Your provider may also take a biopsy of your lung tissue—that is, a small amount of tissue that is removed to look at under a microscope.
Talk to your health care provider if you have a family history of pulmonary fibrosis or have some of the risk factors and symptoms of pulmonary fibrosis.
Treating Pulmonary Fibrosis
Currently, there is no treatment to stop or cure pulmonary fibrosis. Some treatments may help improve the symptoms for a while or slow down the scarring, however. These treatments include medications, oxygen, rehabilitation, and lung transplant.
Living with Pulmonary Fibrosis
Your health care team will help you manage your disease and symptoms. However, living with pulmonary fibrosis means that you must speak for yourself, be prepared for provider visits, and let friends and family help. It may also help for you to contact support groups (such as the Pulmonary Fibrosis Foundation) to find out how others manage their disease.
Each person may have different symptoms and challenges as the disease progresses. Some may have periods where their disease remains stable and their symptoms don’t change. It’s important that you tell your health care team about any changes in your symptoms.
In addition, it’s important to maintain a healthy lifestyle. You can do many things to stay healthy:
- Stop smoking and avoid being around those who smoke.
- Get regular exercise.
- Eat healthy, balanced meals.
- Take your medications.
7 Changes to Improve Your Quality of Life
Get the facts about PF: Myths BUSTED
Myth:
IPF is easy to diagnose
Fact:
IPF symptoms are very similar to symptoms of other lung diseases, such as asthma, COPD, or even heart failure and other diseases that cause scarring in the lungs. It may take years before an accurate diagnosis of IPF is finally made. Half of cases are initially misdiagnosed. That’s why it is so important to track your symptoms, so you can be properly diagnosed. A wide variety of tests need to be done, including extensive occupational, environmental, and social tests, before a confident diagnosis of IPF can be made.
Myth:
I have been diagnosed with IPF, so I only have 3 years to live
Fact:
There is a lot of misleading and false information about IPF on the internet. Many sites claim that an IPF diagnosis means you only have 3 years to live. The truth is that while half of patients diagnosed with IPF may live less than 3 years, half may live more than 3 years. It is hard to predict how IPF will progress in an individual patient: sometimes it progresses quickly, sometimes slowly, or slowly at first then faster later. Some patients have survived more than 10 years with their disease even without receiving medications or having a lung transplant.
Myth:
There is no way to know how my PF will progress
Fact:
Patients with PF typically follow three main paths: stable condition, progressive decline, or rapid decline. The speed of IPF progression varies from patient to patient, but there are some predictors of your individual condition, such as how short of breath you are or how much fibrosis is on your high-resolution computed tomography (HRCT) scan at diagnosis. Close monitoring of your lung function can give a better sense of what the future may hold.
Myth:
Idiopathic Pulmonary Fibrosis is the same as Interstitial Lung Disease
Fact:
Interstitial Lung Disease, or ILD, refers to a large family of lung disorders that tend to have similar abnormalities and symptoms. Some types of ILD can be secondary to another disease, such as rheumatoid arthritis, or a result of an environmental exposure, such as asbestos. Other types have no identifiable cause, in which case they are referred to as idiopathic. One specific type of ILD without a known cause is idiopathic pulmonary fibrosis (IPF). IPF is one type of ILD, but not every type of ILD is IPF (there are more than 200).
Myth:
IPF is only caused by smoking
Fact:
There are no direct links to smoking and getting IPF. In fact, IPF occurs in never-smokers. However, smoking appears to be a risk factor for developing IPF, meaning it can be an indicator to look for IPF. Many patients diagnosed with IPF do have a history of smoking tobacco.
Myth:
There is no treatment for IPF
Fact:
There is no cure for IPF. However, some treatments may help improve symptoms or slow down progression of the disease. Two medicines, pirfenidone (Esbriet®) and nintedanib (Ofev®), have been approved by the U.S. Food and Drug Administration (FDA) for use in patients with IPF. These drugs have been shown to slow the decline of lung function tests. They may decrease hospitalizations due to flare-ups of the disease. There are also therapies, such as supplemental oxygen and pulmonary rehabilitation, that can help manage the symptoms. In the most severe cases, patients with IPF may qualify for a lung transplant.
Myth:
My doctor says I have PF, so that means I have IPF
Fact:
PF is condition of scarring of the lungs. Many types of ILD other than PF or IPF can lead to scarring in the lungs. It is important for your doctor to determine the cause or underlying condition, if identifiable, or if your pulmonary fibrosis is idiopathic because treatments and prognoses for all different forms of ILD will differ.
Pulmonary Fibrosis Myths Busted
Resources
Finding support
It is important to seek the help from local and online support groups to find out how others manage their disease and symptoms. The Pulmonary Fibrosis Foundation website is a valuable resource and can also help you find local support groups and meetings.
Questions to ask your health care provider
You are an important member of your health care team. The more you know, the better you can care for yourself. Ask your provider any questions you have about managing your disease. Questions you may want to ask include:
- Whom should I contact if I have problems with my pulmonary fibrosis?
- When should I start therapy or switch therapies for my fibrosis?
- Are there clinical trials for me to participate in?
- When should I discuss end of life or hospice?
Download and complete our ILD/IPF patient questionnaire to help you initiate a conversation with your doctor about ILD/IPF.
For providers
Patient and Clinician Engagement in Treating Pulmonary Fibrosis Webinar
Understanding where the patient is in their progression of pulmonary fibrosis, as well as any emotional barriers and stigmas, allows clinicians to treat the person rather than just the disease. Meeting patients where they are empowers them to speak directly about the concerns and issues they currently face.
Chest medicine clinicians who have a more objective picture of their patients’ progression can tailor treatments. They can also create an atmosphere where patients are willing to engage and feel motivated to take charge of the management of their pulmonary fibrosis.