Idiopathic Pulmonary Fibrosis

• Risk Factors will discuss family history, lifestyle, or other environmental factors that increase your risk of developing IPF.
• Treatment will discuss medicines, procedures, and lifestyle changes that your doctors may recommend if you are diagnosed with IPF.
• Research for Your Health will discuss how we are using current research and advancing research to better understand and treat IPF.

The risk of developing IPF increases as you age, 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.

• Diagnosis will explain the questions, tests, and procedures that your doctor may use to diagnose IPF.
• Living With will discuss what your doctor may recommend to prevent your IPF getting worse or leading to complications.
• Research for Your Health will discuss how we are using current research and advancing research to better understand and treat IPF.
• Participate in NHLBI Clinical Trials will discuss our open and enrolling clinical studies that are investigating prevention strategies for IPF.

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

Complications of IPF may include the following:

• Depression and anxiety
• Lung cancer
• Pulmonary hypertension
• Respiratory failure
• Sleep problems or disorders

• Diagnosis will discuss tests and procedures used to detect signs of IPF and help rule out other conditions that may mimic IPF.
• Treatment will explain treatment-related complications or side effects.
• Living With will explain what to do if symptoms worsen or come back after treatment.

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

To diagnose IPF, your doctor may have you undergo some of the following tests and procedures.

• High resolution chest CT scan, or HRCT, 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 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 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.

Your doctor may perform some tests and procedures to help rule out other conditions that may cause lung disorders, including the following:

• Blood tests, such as an antibody test to look for signs of an autoimmune disease that can cause lung scarring.
• Bronchoalveolar lavage (BAL) 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.
• Pulmonary function tests 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, if you have a family history of interstitial lung disease or signs of early aging.

• Return to Risk Factors to review family history and genetics, lifestyle, or other environmental factors that increase your risk of developing IPF.
• Return to Signs, Symptoms, and Complications to review common signs and symptoms of IPF.

Your doctor may recommend the following medicines:

• 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.

Your doctor may recommend 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 advanced 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.

• Research for Your Health will discuss how we are using current research and advancing research to better understand and treat people who have IPF.
• Participate in NHLBI Clinical Trials will discuss our open and enrolling clinical studies that are investigating treatments for IPF.
• Living With will explain what your doctor may recommend including life-long healthy lifestyle changes and medical care to prevent your condition from getting worse or to treat complications.

Regular checkups with your doctor may be part of your follow-up and treatment. Tell your doctor if you suddenly experience a worsening of symptoms over a period of days or weeks. This event is called an exacerbation. Sometimes a trigger or other factor may trigger it, but often there is no obvious cause. Exacerbations from IPF can be life threatening and are more common in advanced stages of IPF. People who have an exacerbation from IPF may have repeat episodes.

If your condition worsens, you may benefit from oxygen therapy full time. Some people who have IPF carry portable oxygen when they go out.

Return to Treatment to review possible treatment options for IPF.

Patients who have IPF can have other conditions as well, so it may be necessary to see multiple doctors who specialize in different areas of medical care.

Your doctor may use the following tests and procedures to monitor your condition, and also to determine whether to recommend a lung transplant.

• Chest CT scan to look for lung cancer, or to see how you are responding to treatment or if your condition is getting worse.
• Liver function tests to monitor possible side effects from medicines.
• Pulmonary function tests, which your doctor may recommend every few months.

Your doctor may recommend that you adopt healthy lifestyle changes to increase your overall health and prevent other conditions. This includes:

• Quitting smoking. If you smoke, quit. Also, try to avoid other lung irritants, such as dust, chemicals, and secondhand smoke. Visit Smoking and Your Heart and the National Heart, Lung, and Blood Institute's Your Guide to a Healthy Heart. For free help quitting smoking, you may call the National Cancer Institute's Smoking Quitline at 1-877-44U-QUIT (1-877-448-7848).
• Being physically active. Physical activity can help you maintain your strength and lung function and reduce stress. Try moderate exercise, such as walking or riding a stationary bike. Ask your doctor about using oxygen while exercising.
• Aiming for a healthy weight
• Heart-healthy eating. Also, eating smaller meals more often may relieve stomach fullness, which can make it hard to breathe.

Your doctor may recommend these lifestyle changes as part of a pulmonary rehabilitation program.

Living with IPF may cause fear, anxiety, depression, and stress. Talking about how you feel with your healthcare team may help. Your doctor may recommend:

• Counseling, particularly cognitive behavioral therapy.
• Medicines or other treatments, such as antidepressants, or other treatments that can improve your quality of life.
• Joining a patient support group, which may help you adjust to living with IPF. You can see how other patients manage similar symptoms and their condition. Talk with your doctor about local support groups or check with an area medical center.
• Getting support from family and friends, which can help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you.
• Discussing palliative care, if the disease becomes very advanced. For people who have severe symptoms from IPF, palliative care doctors may help them have a better quality of life with fewer symptoms.

To prevent exacerbations, your doctor may recommend avoiding situations where breathing is more difficult, such as in high altitudes, or when the air quality outside is poor from dust or pollution. Your doctor may treat exacerbations with medicines, such as glucocorticoids, or increased levels of supplemental oxygen.

Return to Signs, Symptoms, and Complications to review complications.