Idiopathic pulmonary fibrosis (IPF) is a serious chronic (long term) disease that affects the tissue surrounding the air sacs, or alveoli, in your lungs. This condition develops when that lung tissue becomes thick and stiff for unknown reasons. Over time, these changes can cause permanent scarring in the lungs, called fibrosis, that makes it progressively more difficult to breathe.
Your risk for IPF is higher if you smoke or have a family history of IPF, and the risk increases with age. The most common symptoms of IPF are shortness of breath and cough. Some people may not have symptoms at first, but symptoms can develop and get worse as the disease progresses.
The way that IPF progresses varies from person to person, and scarring may happen slowly or quickly. In some people, the disease stays the same for years. In other people, the condition quickly gets worse. Many people with IPF also experience what are known as acute exacerbations, where symptoms suddenly become much more serious. Other complications of IPF include pulmonary hypertension and respiratory failure, which happen when the lungs cannot deliver enough oxygen into the bloodstream without support. This prevents the brain and other organs from getting the oxygen they need.
There is currently no cure for IPF. However, certain treatments may slow the progression of IPF and help your lungs work better. This may extend the lifespan and improve the quality of life of people who have the disease.
What causes IPF?
IPF is a type of interstitial lung disease. It is caused by lung tissue becoming thick and stiff and eventually forming scar tissue within the lungs. The scarring, or fibrosis, seems to result from a cycle of damage and healing that occurs in the lungs. Over time, the healing process stops working correctly and scar tissue forms. What causes these changes in the first place is unknown.
In IPF, the scarring makes it difficult to breathe and deliver oxygen from the lungs to the rest of the body. In healthy lungs, oxygen passes easily through the walls of the air sacs, called alveoli, into your capillaries and bloodstream. However, in IPF, the scarring makes the walls of the alveoli thicker. The thickened walls of the alveoli make it harder for oxygen to pass into the blood.
What are the risk factors?
You may have an increased risk for IPF because of your age, family history and genetics, lifestyle habits, or your sex.
- Age: The risk of developing IPF increases as you get older. IPF is diagnosed most often in people who are in their 60s or 70s.
- Lifestyle habits: Smoking is a common risk factor among people who have IPF.
- Sex: IPF is more common among men than women.
- Family history and genetics: Your risk for IPF is higher if a first-degree relative, such as a parent or sibling, has IPF. The specific genes you inherit may make you more likely to develop IPF, especially if those genes contain certain changes, or mutations. To date, mutations in more than 10 different genes have been linked to an increased risk for IPF.
What are the symptoms of IPF?
The most common symptoms are:
- Shortness of breath that gets worse over time. At first, you may be short of breath only during exercise. Over time, you may feel breathless even at rest.
- A dry cough that gets worse. You may have repeated bouts of coughing that you cannot control.
Other symptoms may include:
- Aching muscles and joints
- Clubbing, which is a widening and rounding of the tips of the fingers or toes
- Extreme tiredness
- Gradual, unintended weight loss
- Generally feeling unwell
- Rapid, shallow breathing
To help determine whether you have IPF and rule out other possible causes of lung problems, your doctor may ask about your medical history and possible risk factors.
Your doctor may look for signs of IPF during a physical exam, such as:
- Blue hands and feet from not enough oxygen in the blood
- Clubbing of the fingers or toes
- High-pitched crackles when listening to your lungs
Diagnostic tests and procedures
To diagnose IPF, your doctor may order some of the following tests and procedures.
- High resolution chest CT scan, or HRCT: This is used to take pictures of the inside of your lungs and look for scarring or inflammation. CT scans can also help distinguish between types of lung diseases. For IPF, doctors look for a pattern where the lungs look similar to a honeycomb.
- Lung biopsy: This is used to see if your lung tissue shows signs of inflammation, scarring, or other changes. This procedure is sometimes used to verify the diagnosis of IPF. It usually is done by thoracoscopic surgery, in which small incisions are made and a small camera is used to direct surgical instruments.
- Chest X-ray: This is used to take pictures of the lungs and look for evidence of inflammation or damage such as scarring. An X-ray does not provide as much detail as a high-resolution CT scan.
Tests for other medical conditions
Your doctor may perform some tests and procedures to help rule out other conditions that may cause lung disorders.
- Blood tests, such as an antibodies test: This is used to look for signs of an autoimmune disease that can cause lung scarring.
- Bronchoalveolar lavage (BAL): This is used to look at the types of cells in your lung fluid. Sometimes it may be used when the types of cells can help distinguish between IPF and other lung diseases.
- Lung function tests: This is used to see if you show signs of reduced breathing capacity or abnormal blood oxygen levels. These tests help assess the severity of your lung disease, and they can help monitor whether your condition is stable or worsening over time.
- genetic testing: This is used to see if you have mutations, or changes in your genes, that can raise your risk of IPF. You may need this test if you have a family history of interstitial lung disease or signs of early aging.
How is IPF treated?
You may need the following medicines:
- Nintedanib or pirfenidone can help your lungs work better. They may also help to prevent an acute exacerbation, which is an unexpected and sudden worsening of symptoms over a period of days or weeks, and may prolong survival.
- Antacids may help prevent stomach acid from getting into the lungs from reflux, making IPF worse.
You may need other treatments to treat IPF, including:
- Oxygen therapy to decrease shortness of breath and improve the ability to exercise
- Ventilator support to help with breathing
A lung transplant may be an option for some people who have serious IPF. The major complications of a lung transplant are infection and rejection of the new organ by the body. You will have to take medicines for the rest of your life to reduce the risk of rejection following a lung transplant.