IPF is a type of interstitial lung disease. It is caused by lung tissue becoming thick and stiff and eventually forming scar tissue within the lungs. The scarring, or fibrosis, seems to result from a cycle of damage and healing that occurs in the lungs. Over time, the healing process stops working correctly and scar tissue forms. What causes these changes in the first place is unknown.
To understand IPF it helps to understand How the Lungs Work. In IPF, the scarring makes it difficult to breathe and deliver oxygen from the lungs to the rest of the body. In healthy lungs, oxygen passes easily through the walls of the air sacs, called alveoli, into your capillaries and bloodstream. However, in IPF, the scarring makes the walls of the alveoli thicker. The thickened walls of the alveoli make it harder for oxygen to pass into the blood.
The risk of developing IPF increases as you age, and usually occurs in people older than 50. IPF is diagnosed most often in people who are in their 60s or 70s.
Your risk for IPF is higher if a first-degree relative, such as a parent or sibling, has IPF. The specific genes you inherit may make you more likely to develop IPF, especially if those genes contain certain changes, or mutations. To date, mutations in more than 10 different genes have been linked to an increased risk for IPF.
Your genes can put you at risk for IPF in a few ways.
Scientists have found that mutations in certain genes are common among people who have IPF. Some of these genes help the body make surfactant and mucus, which are important for healthy lung function. One gene, called MUC5B, makes a mucus protein that helps clear harmful substances, such as bacteria, from the lungs. Having a mutated MUC5B gene increases your risk for IPF more than other genes.
Mutations in the TERT and TERC genes are also common in people who have IPF. These genes help protect the DNA in our cells as they divide over our lifespans. The genes do this by producing an enzyme called telomerase. More research must be done to understand why these mutations contribute to the development of IPF.
Learn more about ongoing research we support to better understand how certain gene mutations may cause pulmonary fibrosis.
Smoking is a common risk factor among people who have IPF.
IPF is more common among men than women.
Currently, there are no screening methods to determine who may develop IPF. If you have certain risk factors, especially a family history of IPF, your doctor may recommend you have a pulmonary function test or an imaging test, such as a high-resolution chest CT scan, to look for scarring in the lungs.
The most common signs and symptoms are:
Other signs and symptoms may include:
Your doctor will diagnose IPF based on your symptoms, your medical and family history, your risk factors, and the results from tests and procedures. Idiopathic means that your doctors cannot determine a cause of your disease at the time of diagnosis. They will rule out other medical reasons or conditions that may be causing your symptoms before diagnosing you with IPF. This may be done by doing other tests and talking to specialists.
To help determine whether you have IPF and rule out other possible causes of lung problems, your doctor may ask about your medical history and possible risk factors.
Your doctor may look for signs of IPF during a physical exam, such as:
To diagnose IPF, your doctor may have you undergo some of the following tests and procedures.
Your doctor may perform some tests and procedures to help rule out other conditions that may cause lung disorders, including the following:
There is currently no cure for IPF. Your doctor may recommend medicines, pulmonary rehabilitation, procedures, or other treatments to slow the progression of IPF and help improve your quality of life.
Your doctor may recommend the following medicines:
A lung transplant may be an option for some people who have advanced IPF. The major complications of a lung transplant are infection and rejection of the new organ by the body. You will have to take medicines for the rest of your life to reduce the risk of rejection following a lung transplant.
If you have been diagnosed with IPF, it is important to continue your treatment plan. Talk to your doctor about how often to schedule follow-up care and how to monitor and understand your condition so you know when to get medical help. Your doctor may also recommend lifestyle changes and pulmonary rehabilitation to help you manage the disease.
It is important to know that the progression of IPF is different for each person and cannot be predicted. Managing your condition is best done with the help of a healthcare team that can help improve your quality of life.
Regular checkups with your doctor may be part of your follow-up and treatment. Tell your doctor if you suddenly experience a worsening of symptoms over a period of days or weeks. This event is called an exacerbation. Sometimes a trigger or other factor may trigger it, but often there is no obvious cause. Exacerbations from IPF can be life threatening and are more common in advanced stages of IPF. People who have an exacerbation from IPF may have repeat episodes.
If your condition worsens, you may benefit from oxygen therapy full time. Some people who have IPF carry portable oxygen when they go out.
Return to Treatment to review possible treatment options for IPF.
Patients who have IPF can have other conditions as well, so it may be necessary to see multiple doctors who specialize in different areas of medical care.
Your doctor may use the following tests and procedures to monitor your condition, and also to determine whether to recommend a lung transplant.
Your doctor may recommend that you adopt healthy lifestyle changes to increase your overall health and prevent other conditions. This includes:
Your doctor may recommend these lifestyle changes as part of a pulmonary rehabilitation program.
Living with IPF may cause fear, anxiety, depression, and stress. Talking about how you feel with your healthcare team may help. Your doctor may recommend:
To prevent exacerbations, your doctor may recommend avoiding situations where breathing is more difficult, such as in high altitudes, or when the air quality outside is poor from dust or pollution. Your doctor may treat exacerbations with medicines, such as glucocorticoids, or increased levels of supplemental oxygen.
Return to Signs, Symptoms, and Complications to review complications.
Learn about the following ways the NHLBI continues to translate current research into improved health for people with idiopathic pulmonary fibrosis. Research on this topic is part of NHLBI’s broader commitment to advancing lung diseases scientific discovery.
Learn about some of the pioneering research contributions we have made over the years that have improved clinical care.
In support of our mission, we are committed to advancing IPF research, in part through the following ways.
Learn about exciting research areas the NHLBI is exploring about IPF.
We lead or sponsor many studies on idiopathic pulmonary fibrosis (IPF). See if you or someone you know is eligible to participate in our clinical trials.
After reading our Idiopathic Pulmonary Fibrosis Health Topic, you may be interested in additional information found in the following resources.