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Idiopathic Pulmonary Fibrosis

i-de-o-PA-thic PUL-mo-nar-e fi-BRO-sis

Definition

Idiopathic pulmonary fibrosis (IPF) is a chronic disease. It causes inflammation and fibrosis (scarring) of tissue in the lungs.

Causes

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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Risk Factors

IPF occurs most often in males and people aged 50 years and older. Other factors that may increase your chance of IPF include:

  • Cigarette smoking
  • Viral infection
  • Occupational exposures to dusts containing wood, metal, silica, bacteria, and animal proteins or to aerosol sprays, gases, and fumes
  • Certain medications
  • Gastroesophageal reflux disease (GERD)
  • Other family members with IPF
Symptoms

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 resting
  • Dry cough
  • Gradual weight loss
  • Fatigue
  • Enlargement of the fingertips or sometimes the toes
Diagnosis

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:

Treatment

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

Medication is the main form of treatment. It does not work for everyone. Medications are used to:

  • Reduce inflammation
  • Inhibit immune response
  • Slow the progression of the fibrosis
Gastroesophageal Reflux Disease

If present, GERD will need to be treated. This most often involves the use of medication and lifestyle changes.

Supportive Care
  • Some people may need to receive oxygen. This will help them breathe.
  • A pulmonary rehabilitation program may be needed to improve lung function.
  • Get appropriate vaccinations to prevent lung infection, including a flu shot every year and the pneumonia vaccine.
  • A healthy lifestyle may also help slow the disease. This includes:
    • Not smoking
    • Eating a healthy diet
    • Exercising regularly
    • Resting when needed
  • Lung transplantation may be considered for people with advanced IPF who do not respond to other treatments.
Prevention

There is no proven way to prevent IPF. However, avoiding smoking and wearing masks for work in some occupations may help.

RESOURCES:

American Lung Association
http://www.lung.org

Coalition for Pulmonary Fibrosis
http://www.coalitionforpf.org

CANADIAN RESOURCES

Health Canada
http://www.hc-sc.gc.ca

The Lung Association
http://www.lung.ca

References:

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-pulmonary-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.

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