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Home > Services > Pulmonary & Respiratory Care > Lung Conditions > Idiopathic Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis

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

What Causes IPF?

'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 for IPF

IPF occurs most often in males and people over 50. Other factors below may increase your chance of IPF include:

  • Sex: male
  • Age: 50 and older
  • Cigarette smoking
  • Viral infection
  • Occupational exposures to dusts containing wood, metal, silica, bacteria, animal proteins, aerosol sprays, gases and fumes
  • Medications such as nitrofurantoin, sulfasalazine, amiodarone, propranolol, methotrexate, cyclophosphamide, bleomycin
  • Gastroesophageal reflux disease (GERD)
  • Other family members with IPF

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
  • Clubbing (enlargement of the fingertips or sometimes the toes)

Diagnosing IPF

One or more of the following tests may be performed:

  • Chest X-ray or CT scan —to view the lungs and check for scarring
  • Pulmonary function tests—to measure the size and effectiveness of the lungs
  • Blood test—to tell how well the lungs are taking up oxygen
  • Exercise 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 inflammation
  • Lung biopsy —a small sample of lung tissue is removed and studied; usually required to confirm a diagnosis of IPF

IPF Treatment and Support

IPF has no known cure. Treatment attempts to improve symptoms and slow the disease process by reducing inflammation and scarring. The tissue that is already scarred cannot be returned to normal.

Medication for IPF

The main form of treatment, medication does not work for some people but can:

  • Reduce inflammation
  • Inhibit immune response
  • Slow the progression of the fibrosis

Treating Gastroesophageal Reflux Disease

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

Support Care

  • Oxygen can help some people breathe
  • A pulmonary rehab program may also improve lung function
  • Healthy lifestyle changes may also help slow the disease, including:
    • Healthy diet
    • Regular exercise
    • Rest when needed
    • Not smoking
  • Lung transplantation may be considered for people with advanced IPF who do not respond to other treatment

Preventing IPF

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

Do You Have IPF Symptoms?

Visit the Pulmonary Clinic

Content was created using EBSCO’s Health Library. Edits to original content made by Rector and Visitors of the University of Virginia. This information is not a substitute for professional medical advice.

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Pulmonary Fibrosis in the News

The Pulmonary Fibrosis Foundation has selected UVA to join the foundation’s PFF Care Center Network, becoming one of only 40 such centers in the country.

What does this mean for you? Find out how this honor reflects the quality of our ILD/pulmonary fibrosis program.