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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.

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Risk Factors

IPFoccurs most often in males and people aged 50 and older. Other factors below may increase the risk of getting it:

  • Cigarette smoking
  • Viral infection
  • Occupational exposures to dusts containing wood, metal, silica, bacteria, and animal proteins or to aerosol sprays, gases and fumes
  • Medications such as nitrofurantoin, sulfasalazine, amiodarone, propranolol, methotrexate, cyclophosphamide, bleomycin
  • 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
  • Clubbing (enlargement of the fingertips or sometimes the toes)

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:

  • 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

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 some people. Options may include:

  • Combination of prednisone to reduce inflammation and cytoxan or azathioprine to reduce the body’s immune response
  • To slow the progression of the fibrosis your doctor may recommend:
    • High dose n-acetylcysteine
    • Pirfenidone
    • Interferon-gamma 1b
    • Coumadin
    • Etanercept
    • Bosentan
    • Imatinib
    • Sildenafil
    • Pirfenidone
    • Colchicine
    • Methotrexate
    • Penicillamine
    • Cyclosporine

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 rehab program may also improve lung function.
  • A healthy lifestyle may also help slow the disease. This includes:
    • Not smoking
    • Healthy diet
    • Regular exercise
    • Rest
  • 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.

Revision Information

  • American Lung Association

    http://www.lungusa.org/

  • Coalition for Pulmonary Fibrosis

    http://www.coalitionforpf.org/

  • National Heart, Lung, and Blood Institute

    http://www.nhlbi.nih.gov/

  • The Canadian Lung Association

    http://www.lung.ca/

  • Health Canada

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

  • Bowlby, Lynn. Idiopathic pulmonary fibrosis. In: Ferri’s Clinical Advisor . 9th ed. St. Louis, MO: Mosby; 2007.

  • Epidemiology and risk factors. Coalition for Pulmonary Fibrosis website. Available at: http://www.coalitionforpf.org/ . Accessed January 26, 2004.

  • Facts about idiopathic pulmonary fibrosis. National Heart, Lung, and Blood Institute website. Available at: http://www.nhlbi.nih.gov/ . Accessed January 26, 2004.

  • Facts about pulmonary fibrosis and interstitial lung disease. American Lung Association website. Available at: http://www.lungusa.org/ . Accessed January 26, 2004.

  • Raghu, Ganesh. Intersitial lung disease. In: Cecil Medicine . 23rd ed. Philadelphia, PA: Saunders; 2007.

  • Reporters’ guide to idiopathic pulmonary fibrosis. Coalition for Pulmonary Fibrosis website. Available at: http://www.coalitionforpf.org . Accessed January 27, 2004.

  • Symptoms and diagnosis. Coalition for Pulmonary Fibrosis website. Available at: http://www.coalitionforpf.org/ . Accessed January 26, 2004.

  • Treatment. Coalition for Pulmonary Fibrosis website. Available at: http://www.coalitionforpf.org/ . Accessed January 26, 2004.

  • What is IPF? Coalition for Pulmonary Fibrosis website. Available at: http://www.coalitionforpf.org/ . Accessed January 26, 2004.