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Idiopathic pulmonary fibrosis (IPF)

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Idiopathic pulmonary fibrosis is scarring in the lungs that occurs without a known cause.

Causes of idiopathic pulmonary fibrosis

The exact cause of IPF or why some people get it is yet unknown. Idiopathic means the cause is not known. Genes may play a role in developing it. The disease occurs most often in people between 50 and 70 years old.

Symptoms of IPF

When you have IPF, your lungs become scarred and stiffened. This makes it hard for you to breathe. In most people, IPF gets worse quickly over months or a few years. In others, IPF worsens over a much longer time.

Symptoms may include any of the following:

  • Occasional chest pain
  • Cough, usually dry
  • Shortness of breath during activity—this symptom lasts for months or years, and over time may also occur when at rest

Tests and examinations for IPF

Your provider will do a physical exam and ask about your medical history. You will be asked whether you have been exposed to asbestos or other toxins that affect the lung and if you have been a smoker.

The physical exam may find that you have:

  • Abnormal breath sounds called crackles
  • Bluish skin (cyanosis) around the mouth or fingernails due to low oxygen (with advanced disease)
  • Enlargement and curving of the fingernail bases, called clubbing (with advanced disease)

Tests that help diagnose IPF include the following:

  • Chest CT scan
  • Pulmonary function tests
  • 6-minute walk test
  • Measurements of blood oxygen level (arterial blood gases)
  • Echocardiogram
  • Tests for connective tissue diseases such as rheumatoid arthritis, lupus, or scleroderma
  • Open lung (surgical) lung biopsy

Treatment for IPF

There is no known cure for IPF. Treatment is aimed at relieving symptoms and slowing disease progression:

  • Pirfenidone (Esbriet) and nintedanib (Ofev) are medications that treat IPF. They do not cure the disease, but may help slow lung damage.
  • People with low blood oxygen levels will need oxygen support at home.
  • Lung rehabilitation will not cure the disease, but it can help people exercise with less difficulty breathing.

Making home and lifestyle changes can help manage breathing symptoms. If you or any family members smoke, now is the time to stop.

A lung transplant may be considered for some people with advanced IPF.

Support

You can ease the stress of illness by joining a support group. Sharing with others who have common experiences and problems can help you not feel alone.

These organizations are good resources for information on lung disease:

Outlook for IPF patients

IPF may stay stable for a long time with or without treatment. Most people get worse, even with treatment.

When breathing symptoms become more severe, you and your provider should discuss treatments that prolong life, such as lung transplantation. Also be sure to discuss advance care planning.

Possible complications from IPF

Complications of IPF may include:

  • High blood pressure in the arteries of the lungs
  • Respiratory failure
  • Infections

When to contact a doctor

Call your provider right away if you have any of the following:

  • Breathing that is harder, faster, or shallower (you are unable to take a deep breath)
  • You have to lean forward when sitting to breathe comfortably
  • Frequent headaches
  • Sleepiness or confusion
  • Fever
  • Dark mucus when you cough
  • Blue fingertips or skin around your fingernails