Idiopathic pulmonary fibrosis (IPF) is a chronic disease. It causes inflammation and fibrosis (scarring) of tissue in the lungs.
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.
IPF occurs most often in males and people aged 50 years and older. Other factors that may increase your chance of IPF include:
Over time, the symptoms get worse. This makes daily activities difficult. People with IPF gradually start to have some or all of these symptoms:
You will be asked about your symptoms and medical history. A physical exam will be done.
Your bodily fluids and tissues may be tested. This can be done with:
Images may be taken of your lungs. This can be done with:
Your lung function may be tested. This can be done with:
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 healed.
Medication is the main form of treatment. It does not work for everyone. Medications are used to:
If present, GERD will need to be treated. This most often involves the use of medication and lifestyle changes.
American Lung Association
Coalition for Pulmonary Fibrosis
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 May 9, 2016.
Idiopathic pulmonary fibrosis. American Thoracic Society. Available at: https://www.thoracic.org/patients/patient-resources/resources/idiopathic-pulmonary-fibrosis.pdf. Updated March 2015. Accessed May 9, 2016.
Idiopathic pulmonary fibrosis. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T114208/Idiopathic...lmonary-fibrosis. Updated September 2, 2016. Accessed September 26, 2016.
Pulmonary fibrosis. American Lung Association website. Available at: http://www.lung.org/lung-disease/pulmonary-fibrosis. Accessed May 9, 2016.
Last reviewed June 2016 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.