Pulmonary Fibrosis
What is it?
Pulmonary fibrosis is also known as
interstitial lung disease (ILD), which is a
general term for a variety of chronic lung
disorders. The lungs of a person with
pulmonary fibrosis are stiff because of scarring,
called fibrosis, in the tissue between the air
sacs (the interstitium). The scarred tissue
is unable to transport oxygen. Pulmonary
fibrosis may also be called interstitial pulmonary
fibrosis.
Who gets it?
People who work in coal mines or factories
where there is exposure to metal dusts and
asbestos are more likely to develop pulmonary
fibrosis.
What causes it?
Pulmonary fibrosis occurs when the lung tissue
is damaged, which causes the walls of the lung's
air sacs to become inflamed. With time, this
inflammation leads to scarring in the interstitium,
the tissue between the air sacs. There are several
known causes of the type of lung tissue damage
that results in pulmonary fibrosis. These include
exposure to occupational and environmental
hazards, such as asbestos or metal dusts in mines
or factories. Workers inhale small particles
of these irritants into the lungs. Fumes
found on farms, such as those from moldy hay, can
also cause pulmonary fibrosis. So can a
disease called sarcoidosis. People with this
disease have areas of inflammatory cells called
granulomas in the lungs. Pulmonary fibrosis can
also be caused by the types of radiation used to
treat some cancers; as a complication of diseases
such as rheumatoid arthritis and systemic
sclerosis; as a side effect of some drugs; and
less commonly, through genetic inheritance, called
familial idiopathic pulmonary fibrosis.
When there is no known cause of pulmonary
fibrosis, it is called idiopathic pulmonary
fibrosis (IPF).
What are the symptoms?
The level of symptoms a person experiences
depends on the amount of damage to the lung
tissue. The main symptom is shortness of breath,
which first appears during exercise. As the
condition progresses, and the lungs become more
stiff, the patient may find it impossible to exert
him or herself at all. In its most severe
form, the patient feels short of breath even at
rest. Symptoms may also include a dry cough.
Without treatment, the legs may swell, which is a
signal of heart failure.
How is it diagnosed?
Pulmonary fibrosis is diagnosed by taking a
complete medical history and performing a physical
examination. Your doctor will ask questions
about your employment history, hobbies, drug use,
and health history, including whether you have
arthritis or any diseases that affect the immune
system. Commonly used tests include chest
x-rays, pulmonary (lung) function tests, and blood
tests. Your doctor may also perform a
bronchoalveolar lavage (BAL). With this
test, the doctor uses a flexible fiber optic
viewing tube called a bronchoscope to remove some
cells from your airway. Your throat and
nasal passage will be numbed before the tube is
inserted, and you may be given a sedative to relax
you. The cells are then examined for
any infections or cancers that could be causing
your symptoms. It is important to rule out
any other causes before diagnosing pulmonary
fibrosis. Your doctor may also need to
perform a lung biopsy, where a small piece of lung
tissue is removed for study. This procedure
can also be performed with a biopsy instrument and
the bronchoscope.
What is the treatment?
Many people with pulmonary fibrosis are treated
with corticosteroid drugs. Corticosteroid is
a hormone produced by the adrenal glands that
reduces inflammation. Corticosteroids are
often used in combination with other drugs; your
doctor will recommend the treatment plan that is
right for you. People with breathing
problems are treated with oxygen therapy.
Doctors recommend that patients with pulmonary
fibrosis receive yearly flu vaccinations and a
one-time pneumonia vaccine because these illnesses
can become life-threatening to someone with
existing respiratory problems. Lung
transplants are used only in severe cases.
Self-care tips
You can reduce your chances of developing
pulmonary fibrosis if you make certain lifestyle
changes. Follow a healthy diet and get
plenty of exercise; don't smoke; avoid secondhand
smoke; and avoid spending prolonged periods of
time in areas high in air or industrial pollution.
If you have pulmonary fibrosis, get regular
physical exams to monitor your condition, and take
any prescribed medications according to your
doctor's orders.
This information has been designed as a comprehensive and quick reference
guide written by our health care reviewers. The health information written
by our authors is intended to be a supplement to the care provided by your
physician. It is not intended nor implied to be a substitute for
professional medical advice.
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