Back

Idiopathic Pulmonary Fibrosis

Definition

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.

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.
Healthy Alveoli
Alveoli
Copyright © Nucleus Medical Media, Inc.

Risk Factors

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

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:

Diagnosis

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:

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 returned to normal.

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

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

Support Care

  • 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 smoking
    • Healthy diet
    • Regular exercise
    • Rest when needed
  • Lung transplantation may be considered for people with advanced IPF who do not respond to other treatment.

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

RESOURCES

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.

Revision Information