This September is Global Pulmonary Fibrosis Awareness month. Pulmonary Fibrosis is a serious lung disease, but what exactly is it? Pulmonary, meaning lung, and fibrosis, meaning scar tissue, basically means exactly what the name translates to: scarring in the lungs. Beyond the problems scars present, pulmonary fibrosis impacts how you breathe and get oxygen into the blood. So how can we know more about this largely unspoken disease? We sat down with Dr. Albert Rizzo, Senior Medical Advisor to the American Lung Association, and asked him what you should know about pulmonary fibrosis.

In technical terms, fibrosis means thickening or scarring of the tissue. In this case, the normally thin, lacy walls of the air sacs in the lungs are no longer thin and lacy, but get thick, stiff and scarred, which is also known as fibrotic. With this scarring, the architecture of the lung gets stiffer and is less efficient in the ability to get oxygen into the blood stream. In addition, the stiffness or fibrosis of the tissue makes it more difficult to expand the lungs and breathe.  It takes more energy to breathe and this additional demand of energy leads to shortness of breath with activities that were usually easier to do before. 

There are a number of known causes of pulmonary fibrosis as well as unknown causes, called idiopathic.  Exposure to toxins like asbestos, or coal dust or silica (including workers in the coal mining and sandblasting industry) can lead to pulmonary fibrosis. There are medications known to have a side effect of pulmonary fibrosis (amiodarone, bleomycin, nitrofurantoin, to name a few).  A more common cause of pulmonary fibrosis is that seen in conjunction with a group of diseases known as collagen vascular diseases. This would include systemic lupus, scleroderma, rheumatoid arthritis and Sjogren's syndrome.  There can be familial or hereditary cases of pulmonary fibrosis as well. And as mentioned earlier, there are many cases of pulmonary fibrosis which cannot currently identify a cause and is therefore called "idiopathic" pulmonary fibrosis.

Early symptoms consist of chronic dry cough, and as it progresses, shortness of breath with exertion becomes evident. This can lead to a decrease in exercise tolerance, and this prolonged state of muscles throughout the body going unused can make the patient more easily fatigued. Every person who is diagnosed with pulmonary fibrosis has a unique experience with the disease. Some people stay in stable condition for years, while others may experience rapidly-worsening symptoms and overall health. Although no one can predict your prognosis for certain, you can help yourself by maintaining a healthy lifestyle and working closely with your healthcare team.

It is hard to put a figure on the incidence of pulmonary fibrosis in general because it can co-exist with other conditions such as the collagen vascular diseases mentioned above and is also due to occupational exposures and reactions to certain medications. Idiopathic pulmonary fibrosis, or IPF, is more common than we once thought, with up to 132,000 people affected in the United States. About 50,000 new cases are diagnosed each year. It is more common in men than women, and mostly affects people over 50 years of age.

The Lung Association recommends patients join our Living with Pulmonary Fibrosis and caregivers join our Caring for Pulmonary Fibrosis online support communities to connect with others facing this disease. You can also call the Lung Association's toll-free Lung HelpLine at 1-800-LUNGUSA to talk to a trained respiratory professional or registered nurse who can answer your questions and connect you with support.

It is always important to discuss seeking more options for treatment with your physician. This may lead to second opinions or the possibility of being in a clinical trial. There are a number of clinical trials across the country that can be found at where new drugs are being tested or current drugs are being evaluated in different ways. If you live near a major academic center, it is worth reviewing their website for clinical trials in pulmonary fibrosis for which you might be a candidate.

There is no cure for pulmonary fibrosis. Current treatments are aimed at preventing more lung scarring, relieving symptoms and helping you stay active and healthy. Treatment cannot fix lung scarring that has already occurred.  It is important to be a good communicator with your doctor and making sure that symptoms you have such as cough, shortness of breath are heard and evaluated earlier rather than later. 

  1. What is pulmonary fibrosis?
  2. What causes pulmonary fibrosis?
  3. What is life like for those living with pulmonary fibrosis?
  4. How is common is pulmonary fibrosis?
  5. Are there resources available for patients and caregivers?
  6. Are clinical trials an option?
  7. What is the most important thing everyone should know about pulmonary fibrosis?

Learn more about pulmonary fibrosis.

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