Pulmonary fibrosis is a lung disease that 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 may 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 moderate symptoms that worsen more slowly, over months or years.
Some people may experience a rapid worsening of their symptoms (acute exacerbation), such as severe shortness of breath, that may last for several days to weeks. People who have acute exacerbations may be placed on a mechanical ventilator. Doctors may also prescribe antibiotics, corticosteroid medications or other medications to treat an acute exacerbation.
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 factors — including long-term exposure to certain toxins, certain medical conditions, radiation therapy and some medications.
Occupational and environmental factors
Long-term exposure to a number of toxins and pollutants can damage your lungs. These include:
Some people who receive radiation therapy for lung or breast cancer show signs of lung damage months or sometimes years after the initial treatment. The severity of the damage may depend on:
Many drugs can damage your lungs, especially medications such as:
Lung damage can also result from a number of conditions, including:
Many substances and conditions can lead to pulmonary fibrosis. Even so, in most cases, the cause is never found. Pulmonary fibrosis with no known cause is called idiopathic pulmonary fibrosis.
Researchers have several theories about what might trigger idiopathic pulmonary fibrosis, including viruses and exposure to tobacco smoke. Also, some forms of idiopathic pulmonary fibrosis run in families, and heredity may play a role in idiopathic pulmonary fibrosis.
Many people with idiopathic pulmonary fibrosis may also have gastroesophageal reflux disease (GERD) — a condition that occurs when acid from your stomach flows back into your esophagus. Ongoing research is evaluating if GERD may be a risk factor for idiopathic pulmonary fibrosis, or if GERD may lead to a more rapid progression of the condition. However, more research is needed to determine the association between idiopathic pulmonary fibrosis and GERD.
Factors that make you more susceptible to pulmonary fibrosis include:
Complications of pulmonary fibrosis may include:
This in turn raises pressure within the pulmonary arteries and the lower right heart chamber (right ventricle). Some forms of pulmonary hypertension are serious illnesses that become progressively worse and are sometimes fatal.