Idiopathic pulmonary fibrosis (IPF) is a chronic disease. It causes inflammation and fibrosis (scarring) of tissue in the lungs. It occurs most often in people aged 50-70.
There are various types with similar symptoms, but different response to treatment and outcomes.
Idiopathic means the cause is not known.
Researchers think that IPF is an exaggerated and uncontrolled inflammatory response. This produces the scar tissue. What starts the cycle is not known. Over time, scarring surrounds the thin walled air sacs in the lungs. This makes the tissue thicker and stiffer. As a result, breathing becomes difficult. The lungs gradually lose their ability to pass oxygen to the rest of the body.
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IPFoccurs most often in males and people aged 50 and older. Other factors that may increase your chance of IPF include:
Cigarette smokingViral infectionOccupational exposures to dusts containing wood, metal, silica, bacteria, and animal proteins or to aerosol sprays, gases and fumesMedications such as nitrofurantoin, sulfasalazine, amiodarone, propranolol, methotrexate, cyclophosphamide, bleomycinGastroesophageal reflux disease
Other family members with IPF
Over time, the symptoms get worse. This makes daily activities difficult. People with IPF gradually start to have some or all of these symptoms: Shortness of breath, at first only during or after physical activity, but later also when restingDry coughGradual weight lossFatigueClubbing (enlargement of the fingertips or sometimes the toes)
The doctor will ask about your symptoms and medical history. A physical exam will be done. One or more of the following tests may be performed:
—to view the lungs and check for scarring
Pulmonary function tests
—to measure the size and effectiveness of the lungs
Blood tests—to tell how well the lungs are taking up oxygenExercise test
on treadmill or stationary bicycle—to measure how well the lungs and heart work during physical activity
Bronchoalveolar lavage—fluid is put into the airways and then removed to study the cells and check for signs of inflammationLung biopsy
—a small sample of lung tissue is removed and studied; usually required to confirm a diagnosis of IPF
There is no known cure. The goal of treatment is to improve symptoms and slow the disease process. This is done by reducing inflammation and scarring. The tissue that is already scarred cannot be returned to normal.
Medication is the main form of treatment. It does not work for everyone. Medications are used to:
Reduce inflammationInhibit immune response
Slow the progression of the fibrosis
GERD will need to be treated. This most often involves the use of medication and lifestyle changes.
Some people may need to receive oxygen. This will help them breathe.A pulmonary rehabilitation program may also improve lung function.
A healthy lifestyle may also help slow the disease. This includes:
Not smokingHealthy dietRegular exerciseRest when neededLung transplantation
may be considered for people with advanced IPF who do not respond to other treatment.
There is no proven way to prevent IPF. However, avoiding smoking and wearing masks for work in some occupations may help.
Bowlby, Lynn. Idiopathic pulmonary fibrosis. In:
Ferri’s Clinical Advisor. 9th ed. St. Louis, MO: Mosby; 2007.
Explore idiopathic pulmonary fibrosis. National Heart, Lung, and Blood Institute website. Available at:
http://www.nhlbi.nih.gov/health/health-topics/topics/ipf. Updated September 20, 2011. Accessed June 30, 2015.
Facts about idiopathic pulmonary fibrosis. Coalition for Pulmonary Fibrosis website. Available at:
http://www.coalitionforpf.org/facts-about-idiopathic-pulmonary-fibrosis. Accessed June 30, 2015.
Pulmonary fibrosis. American Lung Association website. Available at:
http://www.lung.org/lung-disease/pulmonary-fibrosis. Accessed June 30, 2015.
Raghu, Ganesh. Intersitial lung disease. In:
Cecil Medicine. 23rd ed. Philadelphia, PA: Saunders; 2007.
Last reviewed June 2015 by Michael Woods, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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