The word idiopathic means unknown, which makes it a fitting name for a disease that’s unfamiliar to many. It’s also unclear why idiopathic pulmonary fibrosis (IPF) develops.

IPF is a lung disease that causes the tissue in your lungs to become stiff. This makes it harder for you to take air in and breathe naturally. Each case of IPF is different, so talk to your doctor for specific details about disease progression.

IPF isn’t the only condition that can make breathing more difficult. Read about the difference between IPF and COPD.

Some cases of pulmonary fibrosis are the result of infections, medications, environmental exposures, and even other diseases. In the majority of IPF cases, doctors aren’t able to identify a cause.

Most people with pulmonary fibrosis have IPF, and a small percentage of people with the disease have a family member with it, too.

Doctors may look at several potential causes when diagnosing pulmonary fibrosis. These can include:

  • pollution and toxins
  • radiation therapy
  • existing conditions
  • medications
  • genetic factors

If they’re unable to find a cause, the disease will be labeled idiopathic. Read more about each of these possible causes, additional risk factors, and the early symptoms of IPF.

Idiopathic pulmonary fibrosis (IPF) causes scarring and stiffness in the lungs. Over time, the scarring will worsen, and the stiffness will make breathing harder.

Common symptoms include:

  • shortness of breath
  • chronic cough
  • weakness
  • fatigue
  • weight loss
  • chest discomfort

Eventually, your lungs may not be able to take in enough oxygen in order to supply your body with the amount it needs. This can ultimately lead to respiratory failure, heart failure, and other health issues. Find out if you’re at risk for IPF and how you can prevent the disease.

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 the lungs gets worse, 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 gets worse. Some people will also experience disease flares, or periods when breathing is more difficult. The damage to the lungs during these flares is not reversible, and you’re unlikely to regain any of the lung function you had before the flare.

Since the signs and symptoms of IPF develop slowly over time, it’s difficult for doctors to diagnose it immediately. The scarring IPF causes also looks similar to scarring caused by other lung diseases.

Doctors may have a hard time telling the difference between IPF and other lung diseases during its early stages. Several tests can be used to confirm IPF and rule out other possible causes.

Tests used to diagnose IPF include:

  • chest X-ray
  • lung function tests
  • pulse oximetry
  • high-resolution computer tomography (HRCT) scan
  • arterial blood glass test
  • exercise testing
  • lung biopsy

About 30,000 to 40,000 new cases of IPF are diagnosed each year in the United States. Were you recently diagnosed with IPF? Begin understanding more about your diagnosis with this guide.

There’s currently no cure for IPF, but treatment options are available to manage and reduce your symptoms. The main goal of medical treatment is 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 medications to control inflammation and reduce lung tissue scaring, and oxygen therapy to help with breathing. A lung transplant may also be needed, but this is often seen as a final treatment step.

Early treatment is vital for people diagnosed with this lung disease. Find out why early treatment can help you manage the symptoms of IPF.

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. But for others, it may take many years before breathing problems are so difficult that they require oxygen.

When lung function becomes severely limited, it can trigger serious complications. These include:

  • heart failure
  • pneumonia
  • pulmonary hypertension
  • pulmonary embolism (blood clot in the lungs).

Existing symptoms can also suddenly get worse after an infection, heart failure, or pulmonary embolism.

Unfortunately, IPF is eventually fatal. The hardened lung tissue may lead to respiratory failure, heart failure, or another life-threatening condition. Talk with your doctor about these seven ways to slow down IPF progression.

IPF typically affects older people between the ages of 50 and 70. Because it affects people later in life, the average life expectancy after a diagnosis is three to five years.

Your own life expectancy with IPF depends on several factors. These include:

  • your age
  • your overall health
  • how quickly the disease progresses
  • the intensity of symptoms

There is no cure for IPF. Researchers are working to raise money for clinical trials that may ultimately lead to a life-saving discovery. Learn more about the outlook and factors that influence how long you may live with the disease.

A large part of managing IPF is learning to adopt healthy lifestyle practices that can reduce symptoms and improve your outlook. These lifestyle changes include:

  • stopping smoking, if you currently do
  • losing weight and maintaining a healthy weight
  • staying on top of all vaccines, medications, and vitamins or supplements
  • using an oxygen monitor to keep your oxygen saturation in optimal range

It’s also a good idea for you to find 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. Management of IPF relies heavily on nonmedical interventions that can improve your quality of life.

If you’ve been diagnosed with IPF, it’s important you continue to seek out ways to care for yourself. Treatment can’t stop the disease, but you can still improve your daily life.

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. Discover other ways you can maintain a good quality of life with IPF.

If you’re experiencing any symptoms of IPF, ask your doctor or healthcare provider about it. The sooner you’re diagnosed, the sooner treatment can begin.

Early treatment can help slow IPF progression. With the right care, it’s still possible to have a good quality of life.