Signature Programs


Join Our Monthly eNewsletter
Subscribe to receive the most current news and updates from the Pulmonary Fibrosis Foundation.

About Idiopathic Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis

Call Our Support Line!

844-825-5733


Feeling confused or overwhelmed about idiopathic pulmonary fibrosis (IPF)? You are not alone. The Pulmonary Fibrosis Foundation is here to help you understand what it means to have IPF. You can always reach us through our Patient Communication Center at 844-825-5733 or by email at pcc@pulmonaryfibrosis.org.

Please remember that this information is a brief overview and is for educational purposes only. It is not intended to be a substitute for professional medical advice. Always consult your health care provider with any questions you may have regarding your specific medical condition.

To find a local idiopathic pulmonary fibrosis support group near you, click here

What is Pulmonary Fibrosis?

The word “pulmonary” means lung and the word “fibrosis” means scar tissue — similar to scars that you may have on your skin from an old injury or surgery. So, in its simplest sense, pulmonary fibrosis (PF) means scarring in the lungs. Over time, the scar tissue can destroy the normal lung and make it hard for oxygen to get into your blood. Low oxygen levels (and the stiff scar tissue itself) can cause you to feel short of breath, particularly when walking and exercising.

Pulmonary fibrosis isn’t just one disease. It is a family of more than 200 different lung diseases that all look very much alike. The PF family of lung diseases falls into an even larger group of diseases called the interstitial lung diseases (also known as ILD), which includes all of the diseases that have inflammation and/or scarring in the lung. Some interstitial lung diseases don’t include scar tissue. When an interstitial lung disease does include scar tissue in the lung, we call it pulmonary fibrosis.

Normal and Impaired Gas Exchange

Idiopathic pulmonary fibrosis (IPF) is a scarring disease of the lungs of unknown cause. To make a diagnosis of IPF, your doctor will perform a thorough history to try to identify potential exposures or other diseases that might lead to scarring of the lung. If a plausible cause is found, then you do not have IPF.

The scarring pattern of IPF is technically called usual interstitial pneumonia (UIP). Your doctor will use detailed X-rays of your lungs called high-resolution computed tomography (HRCT) and sometimes a lung biopsy to look for this pattern.  A diagnosis of IPF requires that your doctor cannot find a cause and the presence of a pattern of UIP on either HRCT or a surgical lung biopsy sample. Possible medications used to treat IPF are discussed on this page.

Although IPF is still considered to be a disease of unknown cause, we do know some factors that increase the risk of getting IPF, including aging (IPF is rare before age 50), cigarette smoking, and having certain genetic predispositions.

There are many forms of ILD that are idiopathic. Most people find these idiopathic ILDs and their abbreviations to be confusing. Here are few of the names and abbreviations you might come across:

 
ABBREVIATION DISEASE
 IPF  Idiopathic pulmonary fibrosis
 Idiopathic NSIP  Idiopathic non-specific interstitial pneumonia
 RB-ILD  Respiratory-bronchiolitis-associatied ILD
 DIP  Desqumative interstitial pneumonia 
 COP or BOOP  Cryptogenic organizing pneumonia
 Sarcoid  Sarcoidosis

No one is certain how many people are affected by PF. One study estimated that idiopathic pulmonary fibrosis affects 1 out of 200 adults over the age of 70 in the United States — that translates to more than 200,000 people living with IPF today. Approximately 50,000 new cases are diagnosed each year.


What are the symptoms of IPF?

Early in the disease, most people with IPF will have no symptoms or might have a bothersome cough. As the disease progresses, breathlessness during exercise and daily activities becomes common. Fatigue, depression, and anxiety are also commonly experienced by people living with IPF.


What causes idiopathic pulmonary fibrosis?

Although the word “idiopathic” means “of unknown cause,” we have learned a lot about the causes of IPF. Former and current smokers are more likely to develop IPF than those who have never smoked. A family history of pulmonary fibrosis is also a risk factor, as are certain genes, such as MUC5B, TERT, TERC, DKC1, RTEL1, AKAP13, DSP, FAM13A, DPP9, and TOLLIP. Some evidence suggests that gastroesophageal reflux (acid reflux, or heartburn), certain viral infections, air pollution, and some exposures in the work place may be risk factors for IPF.


What makes it different from other forms of PF?

IPF is one type of PF. There are many others, some of which have known causes, such as chronic hypersensitivity pneumonitis, connective tissue disease-related ILD, occupational diseases (Pneumoconioses), and drug-induced ILD. In addition to IPF, there are many ILDs of unknown cause – meaning that there are many “idiopathic” ILDs other than IPF. You can read more information about known causes of PF on the About PF page.


What is my prognosis?

Idiopathic pulmonary fibrosis is a progressive disease, which means that fibrosis builds up over time, gradually causing worsening breathlessness and the need for increasing amounts of oxygen. Eventually, lung failure (medically called “respiratory failure”) can develop, which is a life-threatening condition.

It is important for you to know that there is no way to predict how long someone with IPF will live. You may have heard that the average survival of people living with IPF is only “three to five years.” This is an outdated statistic. With earlier diagnosis and better treatments available, many people live much longer than three to five years. Others will develop respiratory failure sooner than three years, with some becoming very ill within months or just a few years after their diagnosis. No one can predict exactly how long you will live with IPF. Everyone is different. Your doctor can give you more detailed information about your prognosis.


How is IPF diagnosed?

When a doctor or other healthcare provider suspects that a patient has ILD, they will perform tests that might include pulmonary function tests, a chest x-ray, blood work, and a high-resolution CT scan. Pulmonary function tests measure how much air the lungs can hold. The more scar tissue the lungs have, the less air they will hold. A doctor will also use a special kind of X-ray of the chest, called a high-resolution computed tomography (HRCT) scan, which gives detailed images of the lung. Healthy lung tissue looks nearly black on a CT scan (also known as CAT scan). Scar tissue and inflammation both appear grey or white.  In many cases, a diagnosis of IPF can be made from these tests. In some cases, a lung biopsy may need to be performed.

 

How is IPF treated?

Pirfenidone (Esbriet®, Pirfenex®, Pirespa®) and Nintedanib (OFEV®) are two drugs approved to treat IPF in many countries around the world. These drugs help to slow the progression of IPF.

Nintedanib (Ofev®): Nintedanib is an anti-fibrotic drug that is approved to treat idiopathic pulmonary fibrosis in the United States, Europe, and Australia. In clinical trials, nintedanib has been shown to slow the decline in lung function in mild-to-moderate idiopathic pulmonary fibrosis. It is taken by mouth twice a day. For more information, please visit ofev.com.

Pirfenidone (Esbriet®, Pirfenex®, Pirespa®): Pirfenidone is an antifibrotic and anti-inflammatory drug approved to treat IPF in the US, Europe, Canada, Asia, and Australia. In clinical trials, pirfenidone has been shown to slow progression of mild-to-moderate idiopathic pulmonary fibrosis. It is taken by mouth three times a day. For more information, please visit esbriet.com.

Lung transplantation is an appropriate treatment for some people living with IPF. Early evaluation for lung transplant is important because the process involves a series of appointments to provide the patient with information about transplantation and to determine if they are an appropriate candidate. 

Pulmonary rehabilitation, supplemental oxygen, smoking cessation, and routine vaccinations (such as influenza and pneumonia vaccination) are important parts of living with IPF.

To learn more about treatment options, click here.


Are there experimental therapies available?

You can search for research studies closest to you on our PFF Clinical Trial Finder at trials.pulmonaryfibrosis.org.

 

What should I do next?

If you have been recently diagnosed with idiopathic pulmonary fibrosis, we suggest that you consider making an appointment with a pulmonologist who has experience caring for patients with IPF. A knowledgeable team of experts will help make sure you receive an accurate diagnosis and the most up-to-date treatments and management recommendations. To assist you in identifying pulmonologists closer to home and developing expertise in the care of patients with PF, the Pulmonary Fibrosis Foundation established the PFF Care Center Network that includes 60 medical centers throughout the United States. You can find a list of sites within the Network here.   

We also recommend that you consider joining a pulmonary fibrosis support group. Connecting with other individuals facing the same illness can help you and your family feel less alone in your journey with idiopathic pulmonary fibrosis. Support groups can supplement the care you receive from your healthcare team by providing emotional support and education. You can find a list of support groups here

Support groups can help those living with idiopathic pulmonary fibrosis

  • Learn about their disease and available treatments;
  • Feel supported by others who are going through the same thing;
  • Learn to navigate the healthcare system more effectively; and
  • Improve coping skills, among other things.

Contact the PFF Patient Communication Center (PCC) by calling 844.TalkPFF (844.825.5733) or email pcc@pulmonaryfibrosis.org with any questions or concerns you may have and to connect you with local resources.

footer_txt_bblBook a PFF Ambassador for your event.   Call 844.TalkPFF >