Important Facts About Idiopathic Pulmonary Fibrosis
What is Idiopathic Pulmonary Fibrosis (IPF)?
IDIOPATHIC PULMONARY FIBROSIS (IPF) is a disease of inflammation that results
in scarring or fibrosis, of the lungs. In time, this fibrosis can build
up to the point where the lungs are unable to provide oxygen to the tissues
of the body.
Doctors use the word "idiopathic" (from the Greek "idio" meaning "peculiar"
or "unusual" and "pathy" meaning "illness") to describe the disease, because
the cause of IPF is unknown. Currently, researchers believe that IPF may
result from either an autoimmune disorder, a condition in which the body's
immune system attacks its own tissues, or the aftereffects of an infection,
most likely a virus. Whatever the trigger is for IPF, it appears to set
off a series of events in which the inflammation and immune activity in
the lungs--and, eventually, the fibrosis processes, too--become uncontrollable.
In a few cases, heredity appears to play a part, possibly making some individuals
more likely than others to get IPF. In studies of patients with IPF, the
average survival rate has been found to be 4 to 6 years after diagnosis.
Those who develop idiopathic pulmonary fibrosis at a young age seem to
have a longer survival.
How Common is IPF?
The exact number of people who develop idiopathic pulmonary fibrosis each
year is not known. It is known, however, that equal numbers of men and
women get the illness and that most cases of IPF are diagnosed when the
patients are between the ages of 40 and 70.
What Are the Symptoms of IPF?
Early symptoms of idiopathic pulmonary fibrosis are usually similar to
those of other lung diseases. Very often, for example, patients suffer
from a dry cough and dyspnea (shortness of breath). As the disease progresses,
dyspnea becomes the major problem. Day-to-day activities such as climbing
stairs, walking short distances, dressing, and even talking on the phone
and eating become more difficult and sometimes nearly impossible. Enlargement
(clubbing) of the fingertips may develop. The patient may also become less
able to fight infection. In advanced stages of the illness, the patient
may need oxygen all the time. IPF can lead to death. Often the immediate
cause is respiratory failure due to hypoxemia, right-heart failure, a heart
attack, blood clot (embolism) in the lungs, stroke, or lung infection brought
on by the disease.
What is the Course of IPF?
Although the course of idiopathic pulmonary fibrosis varies greatly from
person to person, the disease usually develops slowly, sometimes over years.
The early stages are marked by alveolitis, an inflammation of the air sacs
called alveoli, in the lungs. The job of the air sacs is to allow the transfer
of oxygen from the lungs into the blood and the elimination of carbon dioxide
from the lungs and out of the body. As IPF progresses, the alveoli become
damaged and scarred, thus stiffening the lungs. The stiffening makes breathing
difficult and brings on a feeling of breathlessness (dyspnea), especially
during activities that require extra effort.
In addition, scarring of the alveoli reduces the ability of the lungs
to transfer oxygen. The resulting lack of oxygen in the blood (hypoxemia)
may cause increases in the pressure inside the blood vessels of the lungs,
a situation known as pulmonary hypertension. The high blood pressure in
the lungs then puts a strain on the right ventricle, the lower right side
of the heart, which pumps the oxygen-poor blood into the lungs.
How is IPF Diagnosed?
The first suspicion that a person may have idiopathic pulmonary fibrosis
is usually based on the patient's symptoms and medical history. The doctor
will try to confirm or rule out any suspicion by ordering one or more of
the following tests:
A simple chest X-ray is a picture of the lungs and surrounding tissues,
most often taken while the patient is standing up. In an IPF patient, the
X-ray usually reveals shadows, mostly in the lower part of the lungs. In
addition, lung size tends to appear smaller than normal.
Computed Tomography (CT)
A computed tomography scan of the chest is a series of x rays that provide
a view of the lungs that looks almost as if a slice had been made through
the chest. During a CT scan, the patient lies inside a long, oval-shaped
machine that permits x-ray beams to pass through the top, sides, and back
of the body. A computer is used to combine all the pictures taken from
these positions and thus gives the doctor a good look at what's going on
inside the lungs and chest.
When IPF is suspected, the doctor will analyze the patient's blood. A low
level of oxygen in the arterial blood may reveal that the alveoli are not
taking up enough oxygen.
Pulmonary Function Tests
Pulmonary function tests (PFTs) require the patient to breathe into a mouthpiece.
The mouthpiece, in turn, is connected to a machine that measures the amount
of air the patient breathes in and out over a specific period of time.
The results tell the doctor how well the air passages in the lungs are
functioning and how well the lungs are expanding.
Lung washings (bronchoalveolar lavage) are also helpful in arriving at
a diagnosis of IPF. In this procedure, the doctor inserts a long, narrow,
flexible, lighted tube called a bronchoscope down the windpipe and into
the lungs to remove fluid (lavage) and other materials from inside the
lungs. The amounts of certain cells and proteins found in the materials
are measured to determine the stage of the lung disease.
Even if some or all of the results from such tests are abnormal, they
are rarely sufficient to make a specific diagnosis of IPF. The only way
the doctor can confirm a diagnosis of IPF is by examining the lung tissue;
such tissue is usually obtained by an open lung biopsy.
Open Lung Biopsy
In an open lung biopsy, a chest surgeon makes cuts between the ribs in
the chest and removes small pieces of tissue from several places in the
lungs. The material is examined in the laboratory to determine how much
inflammation and fibrosis are in the lungs. It is the only way to confirm
whether the patient has IPF. If IPF is present, the biopsy results are
also the best way to find out how far the disease has progressed and what
the outlook is.
In a patient with no other significant illness, recovery from an open
lung biopsy is relatively quick. The hospital stay is usually 4 to 7 days;
some newer procedures require less surgery, bringing hospital stays to
1 to 3 days.
Can IPF be Treated?
The best chance of slowing the progress of IPF is by treatment as soon
as possible. Most IPF patients require treatment throughout life, usually
under the guidance of a lung specialist. Some major medical centers and
large teaching hospitals do research on the disease and provide consultation
and treatment to patients.
Treatment for idiopathic pulmonary fibrosis may vary a great deal. It
depends on many things, including the age of the patient and stage of the
disease. The aim of treatment is to reduce the inflammation of the alveoli
and stop the abnormal process that ends in fibrosis. Once scar tissue has
formed in the lung, it cannot be returned to normal.
How is IPF Treated?
Drugs are the primary way that IPF is treated. They are usually prescribed
for at least 3 to 6 months. This gives the doctor time to see if a particular
treatment is effective. A combination of tests is used to monitor how well
a particular drug is working. The dose may have to be adjusted so that
the medicine gives the best possible results with the least side effects.
Most side effects are reduced when the dose is made smaller or the drug
is stopped. Commonly used drugs are prednisone and cytoxan. Oxygen administration
and, in special cases, transplantation of the lung are other choices.
A corticosteroid, prednisone, is the most common drug given to patients
with idiopathic pulmonary fibrosis. About 25 to 35 percent of all patients
respond favorably to this medicine. No one knows exactly how corticosteroids
work or why some patients do well on prednisone while others do not. Patients
take prednisone by mouth every morning, starting with a high dose for the
first 4 to 8 weeks. As they improve, they gradually take smaller amounts.
Changes in mood are one of the more common side effects of prednisone;
most patients, however, can handle the mood changes-- anxiety, depression,
or sleeplessness--once they know what is causing the problem. A less common
side effect is a rise in blood-sugar levels.
Cyclophosphamide, also referred to as cytoxan, may be taken together with
prednisone, or instead of it. Like prednisone, cytoxan is swallowed each
day. One of the more serious side effects of cyclophosphamide is leukopenia,
a condition in which the number of white blood cells drops to a dangerously
low level. Leukopenia can be controlled by regularly checking the blood
count and adjusting the dose of cytoxan if necessary.
Azathioprine, penicillamine, chlorambucil, vincristine sulfate, and colchicine
have been used in a few patients with idiopathic pulmonary fibrosis. Their
effectiveness in treating IPF, however, has not been adequately tested.
In addition to treatment with medicine, some patients may need oxygen,
especially when blood oxygen becomes low. This treatment helps resupply
the blood with oxygen. As a result, breathlessness is reduced, the patient
can be more active, and the severity of pulmonary hypertension decreases.
Regular exercise may be useful for patients with IPF. A daily walk or regular
use of a stationary bicycle or treadmill can improve muscle strength and
breathing ability and also increase overall strength. If needed, supplemental
oxygen should be used; sometimes it is the only way a patient is able to
do a reasonable amount of activity.
Lung transplantation, either of both lungs or only one, is an alternative
to drug treatment for patients in the severe, final stages of IPF. It is
most often performed in patients under 60 years of age who do not respond
to any form of treatment. The 1-year survival rate is approximately 60
How Will IPF Affect a Patient's Lifestyle?
Many IPF patients, particularly those in the early stages of the disease,
respond to drug treatment and can continue to go about most of their normal
activities, including working. Some patients with advanced IPF need to
carry oxygen with them.
In addition to getting proper treatment, IPF patients can help themselves
by following the same sensible health measures that everyone should observe.
These include eating a healthy diet, maintaining proper weight, exercising
regularly, and getting enough rest. Above all, IPF patients should not
smoke. Pregnancy is not advisable because the illness puts an extra load
on the heart and lungs.
As with many chronic illnesses, emotional support and psychological
counseling can be of much help to the patient. Most doctors and patients
agree that it is important for both patient and family to be as informed
as possible about IPF. In this way, everyone involved can understand the
illness and apply that information to what is happening in his or her own
Can a Patient Participate in the IPF Research Programs at the National
Institutes of Health (NIH)?
Some people with idiopathic pulmonary fibrosis may be eligible to participate
in an experimental clinical trial at the Warren Grant Magnuson Clinical
Center of the National Institutes of Health in Bethesda, Maryland. Participants
must meet the specific requirements of the study. For more information
on these trials, the patient's doctor should contact:
NIH Patient Referral Service (301) 496-4891
Or the doctor may write to: National Heart, Lung, and Blood Institute
Pulmonary Branch Building 10, Room 6D03 9000 Rockville Pike Bethesda, MD
For additional information, Contact: American Lung Association (Contact
your local chapter in the phone book.) U.S. DEPARTMENT OF HEALTH AND HUMAN
SERVICES Public Health Service National Institutes of Health NIH Publication
No. 93-2997 February 1993
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