Idiopathic pulmonary fibrosis (IPF) is a rare lung condition that causes the tissue in your lungs to become stiff. This makes it harder for you to take air in and breathe naturally.
It’s unclear why IPF develops. The word idiopathic actually means unknown.
Each case of IPF is different, so it’s important to speak with your doctor regarding the specifics about disease progression.
IPF isn’t the only condition that can make breathing more difficult. Get informed about the differences between IPF and chronic obstructive pulmonary disease (COPD).
Doctors may look at several potential causes when diagnosing pulmonary fibrosis. These can include:
- environmental exposures, such as pollution and toxins
- radiation therapy
- other diseases
However, in the majority of pulmonary fibrosis cases, doctors aren’t able to identify a cause. If they’re unable to find a cause, the disease will be labeled idiopathic.
Genetic factors may play some role in the development of pulmonary fibrosis and IPF, but it’s rare for these conditions to occur in more than one member of the same family. Discover other risk factors for IPF here.
IPF causes scarring and stiffness in your lungs. Over time, the scarring will become severe, and the stiffness will make breathing harder.
Common symptoms include:
Eventually, your lungs may not be able to take in enough oxygen to supply your body with the amount it needs. This can ultimately lead to respiratory failure, heart failure, and other health concerns.
However, IPF progresses slowly, and treatments are available. Learn more about the warning signs of IPF.
IPF doesn’t have formal stages, but there are unique periods in the progression of the disease.
When you’re first diagnosed, you may not need oxygen assistance. Soon after, you may discover you need oxygen during activity because you have a hard time catching your breath when walking, gardening, or cleaning.
As scarring in your lungs becomes severe, you’ll likely need oxygen at all times. That includes when you’re active, at rest, and even sleeping.
In the later stages of IPF, high-flow oxygen machines can be used to develop increased levels of oxygen. When you’re away from home, portable machines can provide continuous oxygen.
IPF gradually progresses. Some people will also experience disease flares, or periods when breathing is more difficult. The lung damage that takes place during these flares isn’t reversible, and you’re unlikely to regain any of the lung function you had before the flare.
Taking certain actions can help slow the progression of the disease, though. Check out this list of questions to ask your doctor about IPF progression.
Since the signs and symptoms of IPF develop slowly over time, it’s difficult for doctors to diagnose IPF immediately. Scarring caused by IPF also looks similar to scarring caused by other lung diseases.
Several tests can be used to confirm IPF and rule out other possible causes.
Tests used to diagnose IPF include:
- chest X-ray
- high-resolution computer tomography (HRCT) scan
- exercise testing
- lung function tests
- pulse oximetry, which measures your blood oxygen levels
- blood gas test, which measures your blood levels of oxygen and carbon dioxide
- lung biopsy
According to the Pulmonary Fibrosis Foundation, 50,000 new cases of IPF are diagnosed each year in the United States.
Treatment options are available to help you manage and reduce your symptoms.
The main goals of medical treatment are to reduce lung inflammation, protect lung tissue, and slow the loss of lung function. This will allow you to breathe easier.
The most common treatment options include oxygen therapy and medications. Oxygen therapy helps with breathing, and medications help to control inflammation and reduce lung tissue scarring.
The Food and Drug Administration (FDA) has approved two IPF medications, nintedanib (Ofev) and pirfenidone (Esbriet).
A lung transplant may also be required. Because it’s a major operation, a lung transplant is often seen as the final treatment step. It’s currently the only cure for IPF.
Early treatment is vital for people diagnosed with this lung condition. Learn more about how treatment can help you manage the symptoms of IPF.
An important part of managing IPF is learning to adopt healthy lifestyle practices that can reduce symptoms and improve your outlook and quality of life. These lifestyle changes include:
- stopping smoking, if you currently do
- maintaining a moderate weight, which may require weight loss
- staying on top of all vaccines, medications, and vitamins or supplements
- using an oxygen monitor to keep your oxygen saturation (also known as blood oxygen levels) in optimal range
You may also consider joining a pulmonary rehabilitation support group. These groups, which may be arranged by your doctor’s office or a local hospital, connect you with healthcare professionals and possibly other people who are living with IPF.
Together, you can learn breathing and conditioning exercises as well as techniques for managing stress, anxiety, and the many emotions this diagnosis may cause.
Learning tools for stress management might help prevent worsening symptoms. Support groups can also ease anxiety and feelings of loneliness as you face questions or uncertainty when the disease progresses.
IPF is a progressive disease, which means it will get worse over time. While you can take steps to manage the symptoms, you can’t stop the scarring and lung damage entirely.
For some people, the disease may progress very quickly. For others, it may take many years before breathing problems are so difficult that they require you to use oxygen.
When lung function becomes severely limited, it can trigger serious complications. These include:
The hardened lung tissue that IPF causes may lead to respiratory failure, heart failure, or another life threatening condition.
Existing symptoms can also suddenly get worse after an infection, heart failure, or a pulmonary embolism.
Transplant surgery can help improve your outlook. Other treatments and lifestyle changes can also help slow disease progression.
MedlinePlus says that IPF typically affects people between the ages of 50 and 70 years old. Because it affects people later in life, the average life expectancy after a diagnosis is 3 to 5 years.
If you have IPF, your own life expectancy will depend on several factors. These include:
- your age
- your overall health
- how quickly the disease progresses
- the intensity of symptoms
Researchers are working to raise money for clinical trials that may ultimately lead to a lifesaving discovery. Until a nonsurgical cure for IPF is found, having a lung transplant can help extend your life. Find out more about life expectancy and IPF.