Pulmonary Fibrosis

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General Information About Pulmonary Fibrosis


What is pulmonary fibrosis? Pulmonary fibrosis causes uncontrollable and relentless scarring in the lungs, rendering them stiff and unable to exchange much needed blood oxygen. For largely unknown reasons, normal lung tissue is replaced with scar tissue also known as fibrosis.  This scarring distorts and destroys the normal architecture of the lung and harms the body's ability to take oxygen from the air and deliver it to the blood stream.

There are a large number of known causes of lung scarring or fibrosis.  Some, such as radiation therapy for breast cancer, result in a localized scar that can be seen on an x-ray but in most cases causes little to no breathing problems if it is minimal. Other causes include the body's response to inhaled toxic compounds such as coal (coal workers pneumonconiosis), silica (silicosis) or asbestos (asbestosis); lung complications of certain rheumatologic conditions (e.g. rheumatoid arthritis or scleroderma) or medications (e.g. nitrofurantoin) and allergic reactions to a variety of inhaled allergens (hypersensitivity pneumonitis). Unfortunately, in most patients who develop pulmonary fibrosis, no cause will be identified.  In that case, the disease is termed idiopathic pulmonary fibrosis (IPF).  Idiopathic simply means ‘of unknown cause’ and it results in the progressive scarring of the lungs and ultimately results in respiratory failure and death.

Who is at risk of developing pulmonary fibrosis? Pulmonary fibrosis is most often a disease of older adults, with the majority of patients over 50 years old.  Men are slightly more likely than women and smokers considerably more likely than nonsmokers, to develop the disease.  In about one in five cases, pulmonary fibrosis will run in families and there are a few known genetic changes that can be found in most of the families with the disease. Almost assuredly, it is the combination of one of certain abnormal genes and particular environmental exposures that increases an individual's risk of developing pulmonary fibrosis. 

How is pulmonary fibrosis diagnosed?  Patients with pulmonary fibrosis generally see a physician because of breathlessness or less frequently, a dry cough.  The breathlessness initially occurs with exercise or significant exertion, but can progress to breathlessness with everyday activities or even at rest. Occasionally pulmonary fibrosis is first identified after being seen on a chest x-ray or computerized tomographic (CT) scan that is done for other reasons.  All patients with pulmonary fibrosis will ultimately have a CT of the chest to confirm the presence of the disease, and pulmonary function testing (eg spirometry and diffusing capacity) to measure its severity.  For some patients, the CT scan of the chest will provide adequate information to provide a final diagnosis; many others will need a lung biopsy.  It will be done in one of two ways — either through a bronchoscopy (transbronchial lung biopsy) or through the ribs (surgical lung biopsy) as there are certain microscopic patterns of fibrosis that can give important clues about the underlying cause of the disease and its likelihood of worsening.

How is pulmonary fibrosis treated? No medicines have been shown to improve a patient's long term survival, how they feel (their quality of life), or how much they can do (their functional status). Surgical treatment with lung transplantation is the only treatment that has improved survival. It is important that patients be informed about transplant as an option soon after diagnosis so that the option can be considered and the patient can be evaluated in a timely manner.

While current therapies are inadequate for the vast majority of patients, considerable active research is underway looking for new treatments, and a number of treatment trials are actively enrolling.  While there are no specific medications that have been confirmed to slow or stop disease progression, there are a still a number of things that can be done. For those whose oxygen values are low at rest, with sleep, or during exercise, the use of supplemental oxygen may be beneficial and is often prescribed.  In many patients, pulmonary rehabilitation has been associated with an improvement in quality of life while regular exercise continues. Vaccination for influenza and pneumonia is provided to almost all patients due to increased risk of respiratory failure with these infections.  Patients with pulmonary fibrosis have an increased risk of having sleep apnea and/or gastroesophageal reflux disease (GERD) and should be screened for the conditions early in their treatment.  If identified, both conditions deserve treatment.

Where can I find more information?

Coalition for Pulmonary Fibrosis (www.coalitionforpf.org)
Pulmonary Fibrosis Foundation (www.pulmonaryfibrosis.org)

 Source:  Kevin K. Brown, MD, Chair, ATS Clinical Problems Assembly, and Teresa Barnes, Chair, ATS PAR

Quick Facts About Pulmonary Fibrosis

Every day in America:

  • 128,000 people are suffering from PF
  • 130 people will be diagnosed with PF
  • 110 will die from PF
 (Source: Coalition for Pulmonary Fibrosis)