Idiopathic pulmonary fibrosis (IPF) is a type of lung disease. It causes progressive scarring and stiffness of the lung tissue. It is idiopathic because there is no known cause. People with IPF commonly have shortness of breath and a nonproductive cough. Currently, there is no cure for IPF. This article provides an overview of IPF, including the symptoms, stages, diagnosis, treatment options, and life expectancy.

IPF is a type of progressive interstitial lung disease. This type of lung disease affects the tissues that surround and support the alveoli, or air sacs. Scarring, or fibrosis, of this tissue causes it to stiffen. Fibrosis also makes it harder for the lungs to exchange oxygen and carbon dioxide. This results in shortness of breath and difficulty breathing.

IPF has no known cause. There are other forms of pulmonary fibrosis with underlying causes. However, IPF is the most common type of pulmonary fibrosis. Doctors diagnose IPF in about 50,000 people in the United States each year.

There are no formal stages of IPF. Doctors may refer to the disease as mild, moderate, or severe, but there are no hard definitions for these terms.

Some doctors use a tool called the GAP (gender, age, and physiology) model to help determine the outlook. However, this tool is not always reliable.

Criteria doctors use to try to describe the severity of IPF include:

IPF is an unpredictable disease. The symptoms and their severity vary from person to person.

The course of the disease can also vary, and it can change. For example, some people get severely sick very quickly, while others have mild disease for years. You can also have stable disease that suddenly gets worse.

Some common symptoms of IPF include:

Some people will have flares when their symptoms worsen. If this happens, you may require hospitalization to manage it. These acute exacerbations can be life threatening.

Early diagnosis is important in IPF. It allows doctors to start treatments and make referrals as soon as possible. Contact your doctor on a regular basis for routine health checks. Also, contact your doctor between visits if you notice any unusual symptoms.

IPF has no known cause. Currently, doctors believe that it is probably the result of repeated injuries to the lung tissue over time. 

People who are more likely to develop IPF include:

In addition, IPF is more common in males than females. 

Because there is no clear cause of IPF, there is no definitive way to prevent it. However, basic healthy lifestyle habits can help keep your lungs functioning well. These habits include:

These tips are also important for people who already have IPF.

Diagnosing IPF can be challenging because the signs and symptoms resemble those of many other lung diseases. It is basically a process of ruling out other potential causes. This will require testing, which may involve:

In some cases, doctors may also want to take a fluid or tissue sample from the lungs. This can involve bronchoscopy, a minimally invasive, video-assisted thoracoscopic surgery, or thoracotomy.  

There is no current treatment that will cure IPF. The damage it causes is irreversible. However, some treatments can help improve symptoms and your quality of life.

Treatment options for IPF may include:

Lung transplantation may be an option for some people. Doctors often make this referral early in the disease course. Having a lung transplant has shown benefits in terms of survival.

IPF is a serious lung disease that can become fatal. The average life expectancy after diagnosis is 24–30 months. However, it is extremely variable and unpredictable. Many people live for years with mild disease. Others progress quickly.

There are many factors that go into your outlook. Your doctor is best able to advise you about your prognosis and outlook for the future.

People with IPF should take time to plan for the future. IPF is a progressive disease, and it will be important to address issues ahead of time. This includes making advanced care and end-of-life plans. Working with a counselor, therapist, or social worker can help ease this process.

IPF is progressive, and it can lead to complications such as:

IPF affects everyone differently. The disease will ultimately be fatal. However, it is hard to know how quickly these complications will develop.

IPF is a progressive lung disease that causes scarring of the lung tissue. There is no identifiable cause for IPF. It is also very inconsistent from person to person. This makes it hard for doctors to know how the disease will affect any one person. Although there is currently no cure, there are treatments that can help manage the disease.

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