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Idiopathic Pulmonary Fibrosis
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- 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
- Shortness of breath, at first only during or after physical activity, but later also when resting
- Dry cough
- Gradual weight loss
- Clubbing (enlargement of the fingertips or sometimes the toes)
- 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
- 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
- Interferon-gamma 1b
Gastroesophageal Reflux Disease
- 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
- Lung transplantation may be considered for people with advanced IPF who do not respond to other treatment.
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.
- Reviewer: Michael Woods, MD
- Review Date: 02/2014
- Update Date: 06/20/2013