What Is Pulmonary Fibrosis?
Pulmonary fibrosis is a medical term used to describe scarring and thickening deep inside the lungs. Pulmonary fibrosis is seen as a result of many types of conditions. These conditions have been categorized into a group known as interstitial lung diseases (ILDs). ILDs are also sometimes called interstitial pulmonary fibrosis, although not all interstitial lung diseases will actually cause fibrosis.
Symptoms of Pulmonary Fibrosis
The most common symptoms of pulmonary fibrosis are shortness of breath and a dry, hacking cough that does not go away. Other symptoms that a person may develop over time include:
- Aching muscles and joints
- Gradual, unintended weight loss
- Tiredness
- A general ill feeling (malaise)
- Enlargement of the fingers or toes, which is called clubbing.
As the condition worsens, a person may develop other potentially life-threatening conditions, including:
Pulmonary fibrosis is the end result of long-term inflammation and an abnormal build-up of collagen within the supporting structures of the lung (the interstitial tissue).
There are known causes of the lung damage seen in pulmonary fibrosis. These causes include:
- Long-term exposure to occupational and/or environmental inhalants, such as organic or inorganic dusts, and a variety of irritative or toxic gases.
- Certain antibiotics or chemotherapy drugs
- Radiation, such as that used to treat breast cancer.
There are many other conditions where the cause of pulmonary fibrosis is not known. The most common is
idiopathic pulmonary fibrosis. Other conditions that are associated with pulmonary fibrosis but where the cause is not known include:
- Collagen vascular diseases, such as:
Diagnosing Pulmonary Fibrosis
In order to diagnose pulmonary fibrosis, a healthcare provider will ask a number of questions, perform a physical exam, and recommend certain tests. Some of these tests may allow your healthcare provider to obtain a sample of lung tissue that is then examined under a microscope.
Looking at samples of tissue from several places in your lungs under a microscope is the best way for your healthcare provider to diagnose pulmonary fibrosis. Video-assisted thoracoscopy is typically the procedure that healthcare providers will use to collect the tissue. For this procedure, the healthcare provider inserts a small, lighted tube with a camera (endoscope) into the chest through small incisions between the ribs. The endoscope provides a video image of the lungs and allows the healthcare provider to collect tissue samples. This procedure must be done in the hospital under general anesthesia.
Today, research scientists are beginning to better understand what causes pulmonary fibrosis, and they can diagnose it more quickly. They are also studying several medicines that may slow down the progress of the disease. These efforts should lead to longer and better quality lives for people who have it.
Treatment for pulmonary fibrosis cannot remove scarring that has already happened. Therefore, diagnosing and treating it as early as possible (before a lot of scarring has occurred) is very important.
The main treatment for pulmonary fibrosis is medication, such as
prednisone, to reduce inflammation. Many healthcare providers also add a drug to suppress the body's immune system. These treatments can prevent further scarring and increase survival time in some people, but they don't work for everyone.
Healthcare providers may also recommend oxygen therapy, pulmonary rehabilitations, and/or lung transplantation.
Prognosis for Pulmonary Fibrosis
There is no cure for pulmonary fibrosis, and its effects vary from person to person. In some people, the lung tissue quickly becomes thick and stiff, while in others, the process is much slower. For some people, the condition stays the same for years.
Many people live only about two to five years after the diagnosis. The most common cause of death related to pulmonary fibrosis is respiratory failure. Other causes include:
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