Idiopathic Pulmonary Fibrosis (IPF)
- Article
- 2021-02-03
Idiopathic pulmonary fibrosis (IPF) is a serious chronic disease that affects the tissue around 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 of the lungs called fibrosis that make it increasingly 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 worsen as the disease progresses.
The way IPF progresses varies from person to person, and scarring can occur slowly or quickly. In some people, the disease remains the same for years. In other people the condition diminishes quickly. 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 occur when the lungs cannot bring 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 medications can slow the progression of IPF, which can extend lifespan and improve the quality of life for people with the disease.
Symptoms
The most common signs and symptoms are:
- Shortness of breath that gets worse over time. In the beginning, you may only get short of breath during exercise. Over time, you may even feel breathless at rest.
- A dry cough that gets worse. You may have repeated coughing fits that you cannot control.
Other signs and symptoms can include:
- Painful muscles and joints
- Clubbing, which is a widening and rounding of the tips of the fingers or toes
- Fatigue
- Gradual, unintentional weight loss
- Feeling generally unwell
- Rapid, shallow breathing
Therapy
There is currently no cure for IPF. Your doctor may recommend medications, pulmonary rehabilitation, procedures, or other treatments to slow the progression of IPF and improve your quality of life.
Medicines
Your doctor may recommend the following medications:
- Nintedanib or Pirfenidon to help slow down lung function decline due to IPF. They can also help prevent an acute exacerbation, which is an unexpected and sudden worsening of symptoms over a period of days or weeks, and can prolong survival.
- Antacids to Treat GERD. Treating GERD can help prevent acid from entering the lungs through reflux, making IPF worse.
Other treatments
Your doctor may recommend other treatments to treat IPF, including:
- Oxygen therapy to reduce shortness of breath and improve the ability to exercise.
- Fan support to aid in breathing.
Surgery
A lung transplant may be an option for some people with advanced IPF. The main complications of a lung transplant are infection and the body's rejection of the new organ. You will need to take medication for the rest of your life to reduce the risk of rejection after a lung transplant.
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