Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) occurs when the lung tissue becomes scarred. No one knows what causes this scarring.

IPF is the most common form of interstitial lung disease, a group of diseases that affect the tissue between the airways and bloodstream through both lungs.

The scarring and thickened tissue makes it difficult for the lungs to work properly. As the disease gets worse, it is harder for the lungs to move oxygen into the bloodstream. The body grows weaker and less able without enough oxygen traveling through the blood.

The damage caused by IPF cannot be repaired. Medicines and therapies can sometimes help ease symptoms, slow the disease down and improve quality of life. For some people, a lung transplant might be a possible treatment.


The symptoms of IPF and how quickly the condition worsens may be different for each patient.

The most common symptoms of IPF are shortness of breath and a dry, persistent cough. Other symptoms associated with the condition include:

  • Fast, shallow breathing
  • Gradual weight loss
  • Tiredness
  • Aching joints and muscles
  • Clubbing (widening and rounding of the tips of the fingers or toes)

Causes and Risk Factors

Pulmonary fibrosis is linked to many conditions, but in most cases, the cause is never found. When no cause is known, the condition is called idiopathic pulmonary fibrosis. Researchers are looking into what might cause the disease. Viruses and tobacco smoke are two possible causes. Certain types of the disease seem to run in families.

The condition can affect both men and women and is most often diagnosed in patients between the ages of 50 and 70 years old.


Diagnosis of idiopathic pulmonary fibrosis usually begins with a physical exam. Your doctor will listen your lungs to hear if there are any abnormal sounds that indicate lung disease.

Doctors may use imaging tests, such as chest X-rays and computed tomography (CT) scans, to diagnose the disease.

Pulmonary function tests may be used to find out how much damage has been done and how well your lungs are working.

Doctors may also use more advanced methods to find out the extent of the damage to your lungs.

A bronchoscopy, a procedure that uses a thin instrument passed through the nose or mouth to look at the airway, may be used in different ways. Doctors may use fluid to remove and examine cells from the lung during a bronchoscopy. This procedure is called a bronchoalveolar lavage. A bronchoscopy may also be used to perform a biopsy, which takes a small amount of lung tissue to be tested. Biopsies may also be done separately as a surgery.


There is no cure for idiopathic pulmonary fibrosis. The scarring cannot be repaired. Treatments for the disease may stop further damage and relieve symptoms. The experts in Cedars-Sinai Interstitial Lung Disease program can help.

Treatments include using medicine to reduce the puffiness, or inflammation, of the lungs. These medicines, including corticosteroids, prevent the body’s immune system from causing the inflammation. These medicines may stop further scarring for some patients, such as those whose scarring is related to an autoimmune disease. They don't work for everyone experiencing pulmonary fibrosis, however.

Oxygen therapy may be used if you are not getting enough oxygen into your blood stream. This can help with shortness of breath and allow you to stay active longer. Your doctor may suggest pulmonary rehabilitation. This therapy uses teaching, counseling and exercise to help patients manage their condition.

Gastroesophageal reflux may cause the disease to worsen more quickly, and should be examined and treated by a doctor.

Patients with IPF may need to receive flu and pneumonia vaccinations to reduce their chances of infection.

For some, a lung transplant may be the best option, particularly if you are younger than 65, have no other medical problems and medication isn’t working.

Clinical trials continue to seek new therapies for the disease.