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, and your brain and other organs don't receive 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, and in some people, the condition stays the same for years.
• At present, there is no cure for IPF. 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.
• Some things that may increase your chances of developing IPF include: cigarette smoking, viral infections, exposure to certain environmental pollutants, use of certain drugs, and having gastroesophageal reflux disease.
• Your genes (the basic units of heredity) may also play a role in the development of IPF. Some families have at least two members with IPF.
• The most common symptoms of IPF are shortness of breath and a dry, hacking cough.
• Other symptoms that you may develop over time 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 arterial hypertension, and heart failure.
• To diagnose IPF, your doctor will do a detailed medical history, a physical exam, and several diagnostic tests, including a chest x ray, high-resolution computerized tomography, lung function tests, pulse oximetry, arterial blood gas tests, a skin test for tuberculosis, and exercise testing.
• Looking at samples of tissue from several places in your lungs under a microscope is the best way for your doctor to diagnose IPF. Video-assisted thoracoscopy is the procedure that doctors use most to collect the tissue. Your doctor inserts a small, lighted tube with a camera (endoscope) into your chest through small incisions between your ribs. The endoscope provides a video image of the lungs and allows your doctor to collect tissue samples. This procedure must be done in the hospital under general anesthesia.
• 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.
• Recommended treatments include medicines to reduce inflammation (swelling) in your lungs and/or prevent more scarring, oxygen therapy, pulmonary rehabilitation, and lung transplantation. Pulmonary rehabilitation is now the standard of care for people with ongoing lung disease.
• The main treatment for IPF is medicine, such as prednisone, to reduce inflammation. Many doctors also add a drug to suppress your body's immune system. These treatments can prevent further scarring and increase survival time in some people, but they don't work for everyone.
• Other medicines that may help people with IPF include flu and pneumonia vaccinations; cough medicines or oral codeine; vitamin D, calcium, and a bone-building drug; anti-reflux therapy; and antioxidants.
• If you're still smoking, the most important thing you can do is stop. Avoid secondhand smoke as well.
• Ongoing medical care is important. Treatment by a pulmonologist who specializes in IPF is usually recommended.
• Taking your medicines as your doctor prescribes, making any changes in diet or exercise that your doctor recommends, keeping all of your appointments with your doctor, and enrolling in pulmonary rehabilitation are important.
• Other things you can do for both your physical and mental health include being as active as you can, following a healthy diet, getting plenty of rest, joining a support group, and, as your condition advances, using a wheelchair or motorized scooter and staying busy with activities that aren't physical in nature.