Idiopathic Pulmonary Fibrosis

normal-gas-exchange-impaired-gas-exchange

What is Idiopathic Pulmonary Fibrosis?

Pulmonary fibrosis (PF) is part of a larger group of more than 200 interstitial lung diseases (also known as ILDs) that are characterized by inflammation and/or scarring in the lung. In ILDs, the injury and damage occurs in the walls of the air sacs of the lung, as well as in the tissue and space around these air sacs. When an interstitial lung disease includes scar tissue in the lung, we call it pulmonary fibrosis.

When a person is diagnosed with PF, sometimes a doctor is able to find the cause of the disease. In many cases a doctor can’t find a reason why a person has developed pulmonary fibrosis. When the cause of the disease is not known, the fibrosis may be termed “idiopathic”.

Idiopathic pulmonary fibrosis (IPF) is the most common type of idiopathic interstitial lung disease. But there are multiple forms of ILD that are idiopathic. Your doctor will use detailed X-rays of your lungs called high-resolution computed tomography (HRCT) and sometimes a lung biopsy to look for a specific pattern of scarring on your lungs, called usual interstitial pneumonia (UIP). 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.

It is estimated that IPF 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 Risk Factors for IPF?

Although the word “idiopathic” means “of unknown cause,” we know that there are certain factors that increase the risk of receiving an IPF diagnosis. Current and former smokers are more likely to develop IPF than those who have never smoked. Age may play a role because IPF is rare before the age of 50.

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 certain viral infections, air pollution, and some exposures in the workplace may also be risk factors for IPF. 

There are also conditions, such as gastroesophageal reflux disease (acid reflux, heartburn, or GERD), sleep apnea, or pulmonary hypertension (PH) that are often present in people who are diagnosed with IPF.  These conditions are called “comorbid conditions” or “comorbidities.”  The presence of risk factors and comorbidities provide clues that may be helpful to a physician who suspects that an ILD is present in a patient.

Stethoscope
Woman with PF coughing

What Are the Symptoms of IPF?

IPF shares the same general symptoms as other forms of pulmonary fibrosis. Early in the course of the disease, many people with IPF have no symptoms. Some might have a bothersome cough, or notice that they tire more easily.  As the disease progresses and scarring builds up in the air sacs of the lungs, breathlessness during exercise and daily activities becomes common. People living with PF may also feel fatigued, anxious, or depressed.

How is IPF Diagnosed?

When a doctor or other healthcare provider suspects that a patient has ILD, they will collect information about the patient’s medical and personal history, work and home environment, hobbies, and illness that may be present in the family. This can help a doctor identify exposures or other diseases that might have caused lung injury and scarring. The doctor will also often order 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, and how the lungs are working overall. Scarring can cause the lungs to shrink, and it can also make them stiff and unable to fully expand. This means the lungs are able to hold less air. Scar tissue may also affect the ability of the lungs to transfer oxygen to the bloodstream.

Blood work (serology) can provide information about exposures that may have caused a person’s lung injury, or show that other diseases are present which may be associated with the development of PF. If a cause for the PF, such as an autoimmune disease or environmental exposure, is identified, then the diagnosis is not IPF.

A high-resolution computed tomography (HRCT) scan is a special type of x-ray that shows fine detail of the lung tissue. On a CT scan (also known as CAT scan) healthy lung tissue looks nearly black, and scar tissue and inflammation appear grey or white. A specific scarring pattern called usual interstitial pneumonia (UIP), can help to diagnose IPF when it is seen on an HRCT scan. 

After reviewing the results of all of these tests, a physician can often confirm a diagnosis of IPF.  Sometimes more information is needed and a lung biopsy may be performed.

Patient-and-doctor-examining-HRCT-scan
types-of-ILD

If a Cause Can’t be Found for My Pulmonary Fibrosis, Then is My Diagnosis IPF?

Diagnosing an ILD involves many different types of information, and making an accurate diagnosis can be complicated. Some patients’ disease is easier to diagnose because the cause and type of disease are clear. For other patients a cause cannot be found, and they may not fit easily into any particular category of the disease. In addition to IPF, there are many ILDs of unknown cause – meaning that there are many “idiopathic” ILDs other than IPF. These idiopathic ILDs and their abbreviations can be confusing. This chart shows these different types of idiopathic ILDs.

What is My Prognosis?

Idiopathic pulmonary fibrosis is a progressive disease, which means that fibrosis builds up over time, gradually causing an increase in breathlessness and the need for increasing amounts of oxygen. The progression of IPF is variable, with some people experiencing slower but steady decline in lung function and others developing more rapidly worsening disease. Patients with IPF may have sudden declines in functioning, known as acute exacerbations. 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.

Supplemental-oxygen-tank

How is IPF Treated?

Pirfenidone (Esbriet®, Pirfenex®, Pirespa®) and Nintedanib (OFEV®) are two anti-fibrotic medications approved to treat IPF in many countries around the world. These drugs can help to slow the buildup of scar tissue in the lungs in patients with IPF.

Supplemental oxygen, pulmonary rehabilitation, and management of symptoms are important treatment options for many types of pulmonary fibrosis, depending on how severe the disease is. Smoking cessation and routine vaccinations are important parts of living with pulmonary fibrosis. Lung transplantation may be an appropriate treatment for some people living with IPF.

Are There Experimental Therapies Available?

Some patients choose to participate in clinical trials, which are research studies that explore whether a medical strategy, treatment, or device is safe and effective for use in humans. These studies may also show which medical approaches work best for certain illnesses or groups of people. The purpose of clinical trials is research, so the studies follow strict scientific standards that protect patients and help produce reliable study results. Participation in clinical studies by people living with PF is critical so that we can learn more about the causes of pulmonary fibrosis and find new treatments for PF.

You can learn more about clinical trials in the PFF Clinical Trials Education Center.

Smiling woman and man

What Should I Do Next?

If you have been recently diagnosed with pulmonary fibrosis, consider making an appointment with a pulmonologist who has experience caring for patients with PF. 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 finding pulmonologists nearest to your home who provide expert care to patients with PF, the Pulmonary Fibrosis Foundation established the PFF Care Center Network, which includes 68 medical centers throughout the United States. You can search for the PFF Care Center Network medical center closest to you here.  

We also recommend that you join 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 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 pulmonary fibrosis to:

  • Learn about their disease and available treatments
  • Feel supported by others who share similar experiences living with PF
  • Learn to navigate the healthcare system more effectively
  • Improve coping skills, connect with helpful resources, and more

Contact the PFF Help Center by calling 844.TalkPFF (844.825.5733) or email help@pulmonaryfibrosis.org. We can help answer your questions, discuss your concerns, and connect you with resources.

Educational Materials

Find reliable information and trusted resources that can help you learn about pulmonary fibrosis and live better with PF.

+ VIEW ALL RESOURCES

  • Pulmonary Fibrosis Information Guide

    Our comprehensive guide provides reliable information about pulmonary fibrosis, the diagnostic process, treatment options, and more.
    View Full Details
  • Idiopathic Pulmonary Fibrosis Fact Sheet

    This fact sheet provides an overview of idiopathic pulmonary fibrosis, symptoms, causes, diagnosis, and much more.
    View Full Details
PFF-Help-Center

PFF Help Center

For those living with pulmonary fibrosis, obtaining the most accurate and current information can be a frustrating and challenging task. Let us help you find your answers.