What is Pulmonary Fibrosis?

J. Beam

Pulmonary fibrosis is a disease that causes damage to lung tissue. In medical terms, the word pulmonary refers to the lungs and fibrosis means scarring. Aptly named, pulmonary fibrosis is scarring of the lung tissue. The exact cause of pulmonary fibrosis is unknown, but many factors have been linked to the development of the disease. The symptoms of pulmonary fibrosis mimic many other conditions and thus, it may go undiagnosed in some people.

The human respiratory system, showing the trachea, bronchioles, and lungs.
The human respiratory system, showing the trachea, bronchioles, and lungs.

It is estimated that roughly 40,000 people die from pulmonary fibrosis each year. The disease causes scarring in the lungs, which in turn makes the lung tissue thick and stiff. This reduces the amount of oxygen the lungs can pass onto to the bloodstream and can make it difficult to breathe.

Asbestos exposure increases the risk of pulmonary fibrosis.
Asbestos exposure increases the risk of pulmonary fibrosis.

Factors contributing to the development of pulmonary fibrosis include environmental and occupational pollutants, such as asbestos, chronic illnesses, radiation therapy, and certain medications. Some researchers have linked genetic factors to the disease, but a great deal is still unknown. In some cases, patients have developed pulmonary fibrosis with no apparent cause.

The symptoms of pulmonary fibrosis include shortness of breath, especially during exertion, as well as chronic cough, chest pain and discomfort. Since these symptoms are common of other ailments, pulmonary fibrosis is generally diagnosed based on history of symptoms that have worsened. While diagnostic tests, such as lung function testing, can reveal abnormalities, a lung biopsy can confirm the presence of fibrosis.

At present, there is no known cure for pulmonary fibrosis. Treatment options are limited since the scarring is permanent. The treatment options that do exist may include lung transplant or medication. Pulmonary fibrosis reduces the levels of oxygen in the blood and therefore many patients are treated with oxygen to prevent hypertension and alleviate hypoxia. Other complications can arise from pulmonary fibrosis, including an increased risk for blood clots, which is treated with an anticoagulant.

Patients diagnosed with pulmonary fibrosis should be treated by a pulmonary specialist who has experience with the disease. Life expectancy varies depending on the type and extent of the fibrosis. Though there is no cure for the disease, doctors can alleviate the symptoms with therapy and treatment in many cases.

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