Pulmonary fibrosis occurs when lung tissue becomes damaged and scarred. This thickened, stiff tissue makes it more difficult for your lungs to work properly. As pulmonary fibrosis worsens, you become progressively more short of breath.
The scarring associated with pulmonary fibrosis can be caused by a multitude of factors. But in most cases, doctors can't pinpoint what's causing the problem. When a cause can't be found, the condition is termed idiopathic pulmonary fibrosis.
The lung damage caused by pulmonary fibrosis can't be repaired, but medications and therapies can sometimes help ease symptoms and improve quality of life. For some people, a lung transplant might be appropriate.
Signs and symptoms of pulmonary fibrosis include:
The course of pulmonary fibrosis — and the severity of symptoms — can vary considerably from person to person. Some people become ill very quickly with severe disease. Others have more-moderate symptoms that worsen over months or years.
Pulmonary fibrosis scars and thickens the tissue around and between the air sacs (alveoli) in your lungs. This makes it more difficult for oxygen to pass into your bloodstream. The damage can be caused by many different things — including airborne toxins in the workplace, certain lung diseases and even some types of medical treatments.
Occupational and environmental factors
Idiopathic pulmonary fibrosis: When the cause isn't known
Researchers have several theories about what might trigger idiopathic pulmonary fibrosis, including viruses and exposure to tobacco smoke. And because one type of idiopathic pulmonary fibrosis runs in families, heredity also is thought to play a role.
Bronchioles and alveoli
Within your lungs, the main airways (bronchi) branch off into smaller and smaller passageways, the smallest of which are called bronchioles. At the end of the bronchioles are tiny air sacs (alveoli). ...
Factors that make you more susceptible to pulmonary fibrosis include:
Complications of pulmonary fibrosis may include:
Preparing for your appointment
If your primary care doctor suspects a serious lung problem, you're likely to be referred to a pulmonologist, a doctor who specializes in lung disorders.
What you can do
You might also want to have a friend or family member accompany you to the appointment. Pulmonary fibrosis is a serious and complex disease. A friend or family member can provide emotional support and help remember information that you may have forgotten or missed.
Tests and diagnosis
During the physical exam, your doctor will use a stethoscope to listen carefully to your lungs while you breathe. He or she may also suggest one or more of the following tests.
Pulmonary function tests
Tissue sample may be needed
Treatments and drugs
The lung scarring that occurs in pulmonary fibrosis can't be reversed, and no current treatment has proved effective in stopping the ultimate progression of the disease. Some treatments, though, may improve symptoms temporarily or slow the disease's progress. Others help improve quality of life.
You're most likely to receive oxygen when you sleep or exercise, although some people may use it round-the-clock.
Lifestyle and home remedies
Being actively involved in your own treatment and staying as healthy as possible are essential to living with pulmonary fibrosis. For that reason, it's important to:
Last Updated: 2011-03-15
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