Definition:
Pulmonary Fibrosis is scarring in the
lungs.
Description:
Pulmonary fibrosis develops when the alveoli,
tiny air sacs that transfer oxygen to the blood, become
damaged and inflamed. The body tries to heal the damage
with scars, but these scars collapse the alveoli and
make the lungs less elastic. If the cycle of inflammation
and scarring continues, the lungs become increasingly
unable to deliver oxygen to the blood. Changes in the
lungs can also increase the blood pressure in the pulmonary
artery. This condition, called pulmonary hypertension,
makes the heart work harder and it may fail.
Pulmonary fibrosis can result from many
different lung diseases including sarcoidosis, drug
reactions, autoimmune diseases, environmental allergies
such as Farmer's lung, and exposure to toxic dusts and
gases. Pulmonary fibrosis that develops without a known
cause is called idiopathic pulmonary fibrosis. This
disease is equally common in men and women. It is usually
diagnosed between the ages of 40 and 60.
Causes and Symptoms:
The causes and risk factors vary with
the underlying disease. They may include genetics, environmental
factors, and infections.
The first symptom of pulmonary fibrosis
is usually shortness of breath-at first, during exercise,
but later also while resting. Patients may also have
a dry cough, a rapid heartbeat, or enlargement of the
fingertips and ends of the toes. Some people feel tired
or have a fever, weight loss, muscle or joint pains.
In late stages of the disease, the lack of oxygen in
the blood can give the skin and mucus membranes a blue
tinge known as cyanosis.
Diagnosis:
Patients who are suspected of having Pulmonary
fibrosis are often referred to a lung specialist. Several
tests are usually needed to diagnose this disease and
determine its cause. They include a physical examination,
detailed history of the symptoms, chest x rays, lung
function tests, and blood tests, including a measurement
of the amount of oxygen in the blood. Computed tomography
(CT scan) may give a more detailed picture of the lungs.
Bronchoscopy may be done to examine the air passages
and analyze the cells found deep in the lungs.
Lung biopsies are necessary to diagnose
some diseases. Lung biopsies can be done through a needle
inserted into the chest through the skin, during bronchoscopy,
or as a surgical procedure under general anesthesia.
Treatment:
The treatment of pulmonary fibrosis depends
on the underlying cause. Many diseases are treated by
suppressing inflammation with corticosteroids. Stronger
immune suppressants such as cyclophosphamide (Cytoxan)
or azathioprine (Imuran) may also be tried. Some patients
need supplemental oxygen. A lung transplant may be an
option for incurable diseases. Approximately 60-80%
of patients live for at least two years after the transplant.
There is no good treatment for idiopathic
pulmonary fibrosis. Only 10-20% of patients with this
disease respond to corticosteroids.
Therapies & Treatment:
Anxiety and fear can make breathing difficulties
worse. Some people find that activities such as yoga,
prayer or meditation, music therapy, or biofeedback
help to relax them.
Prognosis:
The prognosis depends on the specific
disease. Some cases may stop progressing or improve,
particularly if the cause can be identified and treated.
Others may develop quickly or slowly into end-stage
lung disease. The course of idiopathic pulmonary fibrosis
is very difficult to predict; however, average survival
is approximately five to seven years.
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