Idiopathic pulmonary fibrosis (IPF) is a serious chronic disease that affects the tissue surrounding the air sacs, or alveoli, in your lungs. This condition occurs 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 make 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 signs and symptoms can develop and get worse as the disease progresses.
The way IPF advances 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 rapidly declines. Many people with IPF also experience what are known as acute exacerbations, where symptoms suddenly become much more severe. Other complications of IPF include pulmonary hypertension and respiratory failure, which happens 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 medicines may slow the progression of IPF, which may extend the lifespan and improve the quality of life for people who have the disease.
Causes
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.
Risk Factors
The risk of developing IPF increases as you get older, and usually occurs in people older than 50. IPF is diagnosed most often in people who are in their 60s or 70s. 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.
Your genes can put you at risk for IPF in a few ways. Smoking is a common risk factor among people who have IPF. IPF is more common among men than women.
The most common signs and 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 signs and symptoms may include:
- Aching muscles and joints
- Clubbing, which is a widening and rounding of the tips of the fingers or toes
- Fatigue
- 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.
Diagnosis of IPF
High resolution chest CT scan 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 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 is usually done by thoracoscopic surgery, where which small incisions are made and a small camera is used to direct surgical instruments.
Chest X-ray 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.
Treatment
There is currently no cure for IPF. Your doctor may recommend medicines, pulmonary rehabilitation, procedures, or other treatments to slow the progression of IPF and help improve your quality of life.
Kinase inhibitors, such as Nintedanib or Pirfenidone, to help slow the decline in lung function from IPF. 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 to help treat GERD. Treating GERD may help prevent acid from getting into the lungs from reflux, making IPF worse.
Oxygen therapy to decrease shortness of breath and improve the ability to exercise. Ventilator support to help with breathing.
