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by Rosenblum LB

Idiopathic Pulmonary Fibrosis

Definition

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

Causes

Idiopathic means the cause is not known.
Something starts uncontrolled inflammation. The inflammation damages tissue and leads to scarring. Over time, the scars surround the thin air sacs in the lungs. They make the sacs thicker and stiffer. Gases like oxygen will have a harder time passing through these sacs. You will have to breathe harder to get enough oxygen. The lungs will worsen until they lose their ability to pass oxygen.
Healthy Air Sacs of Lungs
Alveoli
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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
  • Frequent exposure to dusts containing wood, metal, silica, bacteria, and animal proteins or to aerosol sprays, gases, and fumes
  • Gastroesophageal reflux disease (GERD)
  • Other family members with IPF

Symptoms

Symptoms get worse over time. They will make daily tasks difficult. People with IPF gradually start to have some or all of these symptoms:
  • Shortness of breath during or after physical activity then later also when resting
  • Dry cough
  • Gradual weight loss
  • Fatigue
  • Enlargement of the fingertips, called clubbing, or sometimes the toes

Diagnosis

You will be asked about your symptoms and past health. A physical exam will be done.
Your bodily fluids and tissues may be tested. This can be done with:
  • Blood tests
  • Lung biopsy—a sample of lung tissue is removed and examined in a lab
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 ease symptoms. Options include:

Medicine

Medicine may help to ease some symptoms. A combination of medicine may be used to:
  • Reduce inflammation
  • Slow or decrease the immune response
  • Slow the progress of scarring
Pirfenidone and nintedanib may help to slow fibrosis. This may slow worsening of lung function

Pulmonary Rehabilitation

Diet and exercise can play important roles. Education support can also help to adjust to changes. A pulmonary rehabilitation program may include:
  • Strength and endurance exercise program
  • Educational information and lectures
  • Nutrition education and planning
  • Emotional and social support

Quit and Avoid smoking

Smoking damages lung tissue. It will make symptoms worse. Tools may help to quit smoking. Inhaling smoke from others can be harmful as well.

Other Care

  • Oxygen may be given. This may help to increase oxygen in the body.
  • Vaccines can help to prevent lung infections. Flu shot can be given every year. The pneumonia vaccine can also be helpful.
  • GERD will need to be treated if it is there.
  • Lung transplantation may be needed for severe IPF.

Prevention

There is no proven way to prevent most IPF. Proper protection when working at a high risk job 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

RESOURCES

Explore idiopathic pulmonary fibrosis. National Heart, Lung, and Blood Institute website. Available at: https://www.nhlbi.nih.gov/health-topics/idiopathic-pulmonary-fibrosis. Accessed January 17, 2019.
Idiopathic pulmonary fibrosis. American Thoracic Society. Available at: https://www.thoracic.org/patients/patient-resources/resources/idiopathic-pulmonary-fibrosis.pdf. Accessed January 17, 2019.
Idiopathic pulmonary fibrosis. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T114208/Idiopathic-pulmonary-fibrosis . Updated December 4, 2018. Accessed January 17, 2019.
Pulmonary fibrosis. American Lung Association website. Available at: http://www.lung.org/lung-disease/pulmonary-fibrosis. Accessed January 17, 2019.

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