Key Points

• Pulmonary fibrosis is a condition in which tissue deep in your lungs becomes thick and stiff, or scarred, over time. The development of the scarred tissue is called fibrosis.
• As the tissue becomes thicker, your lungs lose their ability to move oxygen into your bloodstream. As a result, your brain and other organs don't get the oxygen they need.
• Doctors don't know what causes most cases of pulmonary fibrosis. They call these cases idiopathic pulmonary fibrosis (IPF).
• IPF varies from person to person. In some people, the lung tissue quickly becomes thick and stiff. In others, the process is much slower. In some people, the condition stays the same for years.
• IPF has no cure yet. Many people live only 3 to 5 years after diagnosis.
• Doctors don't know what causes IPF. They think that something inside or outside of the lungs attacks the lungs again and again over time. These attacks injure the lungs and cause scarring in the tissue inside and between the air sacs.
• Certain factors may increase your risk for IPF, including cigarette smoking, viral infections, exposure to certain environmental pollutants, the use of certain medicines, and having gastroesophageal reflux disease.
• Your genes (the basic units of heredity) also may play a role in the development of IPF. Some families have at least two members who have IPF.
• The signs and symptoms of IPF develop over time. They may not even begin to appear until the disease has done serious damage to your lungs. The most common symptoms of IPF are shortness of breath and a dry, hacking cough.
• Other symptoms may include rapid, shallow breathing; gradual, unintended weight loss; fatigue (tiredness) or malaise (a general feeling of being unwell); aching muscles and joints; and enlargement of the fingers or toes, which is called clubbing.
• IPF may lead to other serious medical conditions, including a collapsed lung, lung infections, blood clots in the lungs, and lung cancer.
• As your condition gets worse, you may develop other potentially life-threatening conditions, including respiratory failure, pulmonary hypertension, and heart failure.
• Your doctor will diagnose IPF based on your medical history, a physical exam, and the results from tests. Tests can help rule out other causes of your symptoms and show how badly your lungs are damaged.
• IPF is treated with medicines, oxygen therapy, pulmonary rehabilitation (rehab), and lung transplant. The goals of treating IPF are to prevent more lung scarring, relieve your symptoms, maintain your ability to be active and get around, keep you healthy, and improve your quality of life.
• Treatment can't remove scarring that has already happened. Therefore, diagnosing and treating IPF as early as possible (before a lot of scarring has occurred) is very important.
• If you have IPF, lifestyle changes and ongoing care can help you manage the condition. Lifestyle changes include quitting smoking, staying active as much as you can, following a healthy diet, getting plenty of rest, and avoiding situations that can make your symptoms worse.
• Follow your treatment plan as your doctor advises. Take your medicines as prescribed, make any changes in your diet or exercise that your doctor recommends, keep all of your appointments with your doctor, and enroll in pulmonary rehab.