Pulmonary fibrosis (PULL-mun-ary fi-BRO-sis) is a
condition in which tissue deep in your lungs becomes thick and stiff, or
scarred, over time. The development of the scarred tissue is called
fibrosis.
As the lung tissue becomes thicker, your lungs lose
their ability to move oxygen into your bloodstream. As a result, your brain and
other organs don't get the oxygen they need. (See the section on
How
the Lungs Work for more information.)
In some cases, doctors can find out what's causing
the fibrosis. But in most cases, they can't find a cause. They call these cases
idiopathic (id-ee-o-PATH-ick) pulmonary fibrosis (IPF).
IPF is a serious condition. About 200,000 Americans
have it. About 50,000 new cases are diagnosed each year. IPF mostly affects
people who are 50 to 75 years of age.
IPF varies from person to person. In some people,
the lung tissue quickly becomes thick and stiff. In others, the process is much
slower. In some people, the condition stays the same for years.
IPF has no cure yet. Many people live only about 3
to 5 years after diagnosis. The most common cause of death related to IPF is
respiratory failure. Other causes include:
Some people may be more likely to develop IPF
because of their genes (the basic units of heredity). If more than one member
of your family has IPF, the condition is called familial idiopathic pulmonary
fibrosis.
Today, scientists are beginning to understand more
about what causes IPF, and they can diagnose it more quickly. They also are
studying several medicines that may slow the progress of the disease. These
efforts should improve the lifespan and quality of life for people who have
IPF.
How the Lungs Work
The air that you breathe in through your nose or
mouth travels down through your trachea (windpipe) into two tubes in your lungs
called the bronchial tubes, or airways.
The airways are shaped like an upside-down tree with
many branches. The windpipe is the trunk. It splits into two bronchial tubes,
or bronchi. Thinner tubes called bronchioles branch out from the bronchi.
The bronchioles end in tiny air sacs called alveoli.
These air sacs have very thin walls, and small blood vessels called capillaries
run through them. There are about 300 million alveoli in a normal lung.
When the air that you've just breathed in reaches
these air sacs, the oxygen in the air passes through the air sac walls into the
blood in the capillaries. The blood then flows into larger veins, which carry
it to the heart. Your heart then pumps the oxygen-rich blood to all your body's
organs. These organs can't function without an ongoing supply of oxygen.
The animation below shows how the lungs work. Click
the "start" button to play the animation. Written and spoken explanations are
provided with each frame. Use the buttons in the lower right corner to pause,
restart, or replay the animation, or use the scroll bar below the buttons to
move through the frames.
The animation shows how the lungs
inhale oxygen and transfer it to the blood. It also shows how carbon dioxide (a
waste product) is removed from the blood and exhaled.
In idiopathic pulmonary fibrosis, scarring begins in
the air sac walls and the spaces around them. It makes the walls of the air
sacs thicker. As a result, oxygen can't move from the air sacs into the
bloodstream.
For more information about lung function, see the
Diseases and Conditions Index
How
the Lungs Work article.
Doctors don't know what causes idiopathic pulmonary
fibrosis (IPF). They think that something inside or outside of the lungs
attacks the lungs again and again over time.
These attacks injure the lungs and cause scarring in
the tissue inside and between the air sacs. This makes it harder for oxygen to
pass through the air sac walls into the bloodstream.
Doctors think the following things may increase your
risk for IPF:
Cigarette smoking
Viral infections, including Epstein-Barr virus
(which causes mononucleosis), influenza A virus, hepatitis C virus, HIV-a
virus, and herpes virus 6
Exposure to environmental pollutants, including
inorganic dust (silica and hard metal dusts), organic dust (bacteria and animal
proteins), and gases and fumes
The use of certain medicines, including:
Nitrofurantoin and sulfasalazine, which are
antibiotics used mostly to treat urinary tract infections, ulcerative colitis,
and rheumatoid arthritis
Medicines that keep your heartbeat regular,
such as amiodarone or propranolol
Chemotherapy or cancer medicines, such as
methotrexate, cyclophosphamide, and bleomycin
Genetics also may play a role in causing IPF. Some
families have at least two members who have IPF.
Scientists have found that 9 out of 10 people who
have IPF also have gastroesophageal reflux disease (GERD). GERD is a condition
in which acid from your stomach backs up into your throat.
As a result, some scientists think that some people
who have GERD may breathe in tiny drops of acid from their stomachs on a
regular basis. The acid may then injure the lungs and lead to IPF. More
research must be done to confirm this theory.
What Are the Signs and Symptoms of Idiopathic
Pulmonary Fibrosis?
The signs and symptoms of idiopathic pulmonary
fibrosis (IPF) develop over time. They may not even begin to appear until the
disease has done serious damage to your lungs. Once they occur, they're likely
to get worse over time.
The most common signs and symptoms are:
Shortness of breath. This usually is the main
symptom. At first, you may be short of breath only during exercise. Over time,
you're likely to feel breathless even at rest.
A dry, hacking cough that doesn't get better.
Over time, you may have repeated bouts of coughing that you can't control.
Other signs and symptoms that you may develop over
time include:
Rapid, shallow breathing
Gradual, unintended weight loss
Fatigue (tiredness) or malaise (a general
feeling of being unwell)
Aching muscles and joints
Clubbing, which is the widening and rounding of
the tips of the fingers or toes
IPF may lead to other medical conditions, including
collapsed lung, lung infections, blood clots in the lungs, and lung cancer.
As your condition gets worse, you may develop other
potentially life-threatening conditions, including respiratory failure,
pulmonary
hypertension, and
heart
failure.
How Is Idiopathic Pulmonary Fibrosis
Diagnosed?
Idiopathic pulmonary fibrosis (IPF) causes the same
kind of scarring and symptoms as some other lung diseases. This makes it hard
to diagnose.
Seeking medical help, preferably from a
pulmonologist, as soon as you have symptoms is important. A pulmonologist is a
doctor who specializes in diagnosing and treating lung problems.
Your doctor will diagnose IPF based on your medical
history, a physical exam, and the results from tests. Tests can help rule out
other causes of your symptoms and show how badly your lungs are damaged.
Medical History
Your doctor may ask about:
Your age
Your history of smoking
Things in the air at your job or elsewhere that
could irritate your lungs
Your hobbies
Your history of legal and illegal drug use
Other medical conditions that you have
Your family's medical history
How long you've had symptoms
Diagnostic Tests
No single test can diagnose IPF. Your doctor may
recommend several of the following tests.
Chest X Ray
A
chest
x ray is a painless test that creates a picture of your heart and lungs. It
can show shadows that suggest scar tissue. However, many people who have IPF
have normal chest x rays at the time they're diagnosed.
HRCT can show scar tissue and how much lung damage
you have. This test can help your doctor spot IPF at an early stage or rule it
out. It also can help your doctor decide how likely you are to respond to
treatment.
Lung Function Tests
Your doctor will do a breathing test called
spirometry
(spi-ROM-eh-tre) to find out how much lung damage you have. A spirometer is a
device that measures how much air you can blow out of your lungs after taking a
deep breath. It also measures how fast you can breathe the air out.
If your lungs have a lot of scarring, you will not
be able to move a normal amount of air out of them.
Pulse Oximetry
For this test, your doctor attaches a small device
to your finger or earlobe. This device measures how much oxygen is in your
bloodstream.
Arterial Blood Gas Test
For this test, your doctor takes a small amount of
blood from an artery in your arm, usually in your wrist. The blood is then
checked for oxygen and carbon dioxide levels.
This test is a more accurate way to check the level
of oxygen in your bloodstream. Your blood also can be tested to see whether an
infection is causing your symptoms.
Skin Test for Tuberculosis
For this test, your doctor injects a small amount of
a substance that reacts to tuberculosis (TB) under the top layer of skin on one
of your arms. If you have a positive reaction, a small hard lump will develop
at the injection site 48 to 72 hours after the test. This test is done to rule
out TB.
Exercise Testing
Exercise testing is done to find out how well your
lungs move oxygen and carbon dioxide in and out of your bloodstream when you're
active. During this test, you walk or pedal on an exercise machine for a few
minutes.
An
EKG
(electrocardiogram) monitors your heart rate, a blood pressure cuff checks your
blood pressure, and a pulse oximeter attached to a finger or ear lobe shows how
much oxygen is in your blood.
Your doctor may place a catheter (a flexible tube)
in an artery in one of your arms to draw samples of your blood. These samples
will provide a more accurate measure of the oxygen and carbon dioxide in your
blood.
Your doctor also may ask you to breathe into a tube
that measures the amount of oxygen you've taken in and carbon dioxide you're
breathing out.
Lung Biopsy
For a lung biopsy, your doctor will take samples of
lung tissue from several places in your lungs and then examine them under a
microscope. A lung biopsy is the best way for your doctor to diagnose IPF.
This procedure can help your doctor rule out other
causes of your condition, such as
sarcoidosis,
cancer, or infection. Lung biopsy also can show your doctor how far your
condition has advanced.
Doctors use several different procedures to get lung
tissue samples.
Video-assisted thoracoscopy
(thor-ah-KOS-ko-pe). This is the most common procedure used to get
lung tissue. Your doctor inserts a small, lighted tube with a camera
(endoscope) into your chest through small incisions (cuts) between your ribs.
The endoscope provides a video image of the lungs
and allows your doctor to collect samples of tissue. This procedure must be
done in a hospital, and you'll be given medicine to temporarily put you to
sleep.
Bronchoscopy (bron-KOS-ko-pee). For
a bronchoscopy,
your doctor passes a thin, flexible tube through your nose (or sometimes your
mouth), down your throat, and into your airways. At the bronchoscope's tip are
a light and a mini-camera, so your doctor can see your windpipe and
airways.
Your doctor then inserts a forceps through the tube
to collect tissue to examine. You'll be given medicine to make you relaxed and
sleepy during the procedure.
Bronchoalveolar lavage (BRONG-ko-al-VE-o-lar
lah-VAHZH). During bronchoscopy, your doctor may inject a small amount
of salt water (saline) through the tube into your lungs. This fluid washes the
lungs and helps bring up cells from the area around the air sacs for
examination under a microscope.
Thoracotomy (thor-ah-KOT-o-me). For
this procedure, your doctor removes a few small pieces of lung tissue through
an incision in the chest wall between your ribs. Thoracotomy is done in a
hospital, and you will be given medicine to temporarily put you to sleep.
How Is Idiopathic Pulmonary Fibrosis Treated?
Idiopathic pulmonary fibrosis (IPF) is treated with
medicines, oxygen therapy, pulmonary rehabilitation (rehab), and
lung
transplant.
The goals of treating IPF are to prevent more lung
scarring, relieve your symptoms, maintain your ability to be active and get
around, keep you healthy, and improve your quality of life.
Treatment can't remove scarring that has already
happened. As a result, diagnosing and treating IPF as early as possible, before
a lot of scarring has taken place, is very important.
Treatment usually is based on your age, medical
history, any medical problems you may have, and how much the IPF has
advanced.
Medicines
The main treatment for IPF is medicine to reduce
inflammation. Many doctors also add a medicine to suppress your body's immune
system. These treatments can prevent further scarring and increase survival
time in some people, but they don't work for everyone.
Prednisone
The anti-inflammatory medicine that most doctors
prescribe is high-dose prednisone, a corticosteroid. You usually take
prednisone by mouth every day. However, your doctor may give it to you through
a needle or tube inserted into a vein in your arm for several days. After that,
you usually take it by mouth.
Because prednisone can cause serious side effects,
your doctor may prescribe it for only 3 to 6 months at first. Then, if it works
for you, your doctor may reduce the dose over time and keep you on it for a
longer time.
Most people who take high-dose prednisone for a long
time can have side effects, including
insomnia,
weight gain, acne, and irritability. Using prednisone for a long time also can
lead to other conditions, including:
Glaucoma (a serious eye condition that can lead
to blindness).
Anxiety or depression.
Osteoporosis (thinning of the skin and bones).
Adrenal gland insufficiency (a condition in
which the adrenal glands don't produce enough of certain hormones). This
condition should be treated by an endocrinologist. This is a doctor who
specializes in diagnosing and treating a number of endocrine problems,
including adrenal gland problems.
Prednisone also can cause conditions such as
diabetes and glaucoma to get worse.
Other Medicines
Many doctors prescribe a second medicine with
prednisone.
Azathioprine. This medicine affects
your immune system. Most patients take it by mouth every day. Because it can
cause serious side effects, your doctor may prescribe it with prednisone for
only 3 to 6 months.
Then, if you don't have serious side effects and the
drug combination seems to help you, your doctor may keep you on it long term.
The most common side effects of azathioprine include:
Nausea (feeling sick to your stomach), vomiting,
diarrhea, and fever and chills
Anemia
(a low red blood cell count) and low platelet and white blood cell counts
Liver problems
Pancreatitis or lymphoma (rarely)
Cyclophosphamide. This is another
immune system suppressant that doctors use to treat IPF. They usually add it to
low doses of prednisone for patients who are getting worse while taking
prednisone alone. Many patients who can't take prednisone take cyclophosphamide
alone.
Most people take cyclophosphamide by mouth every
day. Some IPF patients get it for 3 to 5 days through a needle that's inserted
into a vein in the arm. After that, they take it by mouth every day. You
usually start on a low dose that's increased over time. It may take 3 to 6
months before you see any benefits from cyclophosphamide.
The most common side effect of cyclophosphamide is a
decrease in the number of blood cells that you have. This increases your
chances for infection. Your doctor may recommend
blood
tests before, during, and after your treatment to see how this medicine
affects your blood cells.
Other side effects can include:
Infertility in both men and women.
Nausea, diarrhea, and fatigue (tiredness).
Hair loss.
Bladder irritation. Some people who have taken
cyclophosphamide for more than 2 years have developed bladder cancer. If you
take cyclophosphamide, you should drink at least 8 glasses of water a day. Your
doctor should test your urine at least monthly.
Other medicines that may help people who have IPF
include the following:
Flu and
pneumonia
vaccines may help prevent infection and keep you healthy.
Cough medicines or oral codeine may relieve
coughing.
Vitamin D, calcium, and a bone-building medicine
may help prevent bone loss if you're taking prednisone or another
corticosteroid.
Anti-reflux therapy may help control
gastroesophageal reflux disease.
New Medicines Being Studied
Researchers, like those in the Idiopathic Pulmonary
Fibrosis Network, are studying new treatments for IPF. With the support and
guidance of the National Heart, Lung, and Blood Institute, the researchers who
are part of this network continue to look for new IPF treatments and therapies.
Some of these researchers are looking at medicines
that may reduce inflammation and/or prevent or reduce scarring in IPF.
Oxygen Therapy
When the amount of oxygen in your blood gets low,
you may need oxygen therapy. Oxygen therapy can help reduce shortness of breath
and allow you to be more active.
Oxygen usually is given through nasal prongs or a
mask. At first, you may need it only during exercise and sleep. As your
condition gets worse, you may need it all the time.
Pulmonary Rehabilitation
Pulmonary rehab is now the standard of care for
people who have ongoing lung disease. Rehab usually involves treatment by a
team of specialists in a specialized clinic. The goal is to teach you how to
manage your condition and function at your best.
Services usually include:
Physical conditioning training
Breathing exercises and retraining, so that it
takes less energy for you to breathe
Anxiety, stress, and depression management
Nutritional counseling
Support groups
Lung Transplant
Early referral for a lung transplant may be
recommended if you:
Are younger than 65
Have no other medical problems
Are not being helped by medicines
Single lung transplant can improve your quality of
life and help you live longer.
Complications can include rejection by the body of
the transplanted lung and infection. You may have to take medicines for the
rest of your life to reduce the chances that your body will reject the
transplanted lung.
Because the supply of donor lungs is limited, asking
for an evaluation for a transplant as soon as possible is important.
Living With Idiopathic Pulmonary Fibrosis
There's no cure for idiopathic pulmonary fibrosis
(IPF) yet. Your symptoms may get worse over time. As your symptoms worsen, you
may not be able to do many of the things that you did before you had IPF.
However, lifestyle changes and ongoing care can help
you manage your condition.
Lifestyle Changes
If you're still smoking, the most important thing
you can do is stop. Talk to your doctor about programs and products that can
help you quit. Also, try to avoid secondhand smoke. Ask family members and
friends not to smoke in front of you or in your home, car, or workplace.
Staying active can help with both your physical and
mental health. Physical activity can help you maintain your strength and lung
function and reduce stress. Try moderate exercise, such as walking or riding a
stationary bike. Ask your doctor about using oxygen while exercising.
As your condition advances, use a wheelchair or
motorized scooter, or stay busy with activities that aren't physical in
nature.
You should also follow a healthy diet. A healthy
diet includes a variety of fruits, vegetables, and whole grains.
It also includes lean meats, poultry, fish, beans,
and fat-free or low-fat milk or milk products. A healthy diet is low in
saturated fat, trans fat, cholesterol, sodium (salt), and added
sugar.
Eating smaller, more frequent meals may relieve
stomach fullness, which can make it hard to breathe. If you need help with your
diet, ask your doctor to arrange for a dietitian to work with you.
Getting plenty of rest can increase your energy and
help you deal with the stress of living with a serious condition like IPF.
Maintain a positive attitude. Practicing relaxation
techniques may help you do this. It also may help you avoid excessive oxygen
intake caused by tension or overworked muscles.
Avoid situations that can make your symptoms worse,
such as traveling by air or living at or traveling to high altitudes where the
air is thin and the amount of oxygen in the air is low.
Ongoing Care
If you have IPF, you will need ongoing medical care.
Treatment by a pulmonologist who specializes in IPF usually is recommended.
These specialists often are located at major medical centers.
Treatment may relieve your symptoms and even slow or
stop the fibrosis (scarring). Follow your treatment plan as your doctor
advises. For example:
Take your medicines as your doctor prescribes
Make any changes in diet or exercise that your
doctor recommends
Keep all of your appointments with your doctor
Enroll in pulmonary rehabilitation (rehab)
As your condition worsens, you may need oxygen
therapy full time. Some people who have IPF carry portable oxygen when they go
out.
Emotional Issues and Support
Living with IPF may cause fear, anxiety, depression,
and stress. It's important to talk about how you feel with your health care
team. Talking to a professional counselor also can help. If you're feeling very
depressed, your health care team or counselor may prescribe medicines to make
you feel better.
Joining a patient support group may help you adjust
to living with IPF. You can see how other people who have the same symptoms
have coped with them. Talk to your doctor about local support groups or check
with an area medical center.
Support from family and friends also can help
relieve stress and anxiety. Let your loved ones know how you feel and what they
can do to help you.
Key Points
Pulmonary fibrosis is a condition in which
tissue deep in your lungs becomes thick and stiff, or scarred, over time. The
development of the scarred tissue is called fibrosis.
As the tissue becomes thicker, your lungs lose
their ability to move oxygen into your bloodstream. As a result, your brain and
other organs don't get the oxygen they need.
Doctors don't know what causes most cases of
pulmonary fibrosis. They call these cases idiopathic pulmonary fibrosis (IPF).
IPF varies from person to person. In some
people, the lung tissue quickly becomes thick and stiff. In others, the process
is much slower. In some people, the condition stays the same for years.
IPF has no cure yet. Many people live only 3 to
5 years after diagnosis.
Doctors don't know what causes IPF. They think
that something inside or outside of the lungs attacks the lungs again and again
over time. These attacks injure the lungs and cause scarring in the tissue
inside and between the air sacs.
Certain factors may increase your risk for IPF,
including cigarette smoking, viral infections, exposure to certain
environmental pollutants, the use of certain medicines, and having
gastroesophageal reflux disease.
Your genes (the basic units of heredity) also
may play a role in the development of IPF. Some families have at least two
members who have IPF.
The signs and symptoms of IPF develop over time.
They may not even begin to appear until the disease has done serious damage to
your lungs. The most common symptoms of IPF are shortness of breath and a dry,
hacking cough.
Other symptoms may include rapid, shallow
breathing; gradual, unintended weight loss; fatigue (tiredness) or malaise (a
general feeling of being unwell); aching muscles and joints; and enlargement of
the fingers or toes, which is called clubbing.
IPF may lead to other serious medical
conditions, including a collapsed lung, lung infections, blood clots in the
lungs, and lung cancer.
As your condition gets worse, you may develop
other potentially life-threatening conditions, including respiratory failure,
pulmonary
hypertension, and
heart
failure.
Your doctor will diagnose IPF based on your
medical history, a physical exam, and the results from tests. Tests can help
rule out other causes of your symptoms and show how badly your lungs are
damaged.
IPF is treated with medicines, oxygen therapy,
pulmonary rehabilitation (rehab), and
lung
transplant. The goals of treating IPF are to prevent more lung scarring,
relieve your symptoms, maintain your ability to be active and get around, keep
you healthy, and improve your quality of life.
Treatment can't remove scarring that has already
happened. Therefore, diagnosing and treating IPF as early as possible (before a
lot of scarring has occurred) is very important.
If you have IPF, lifestyle changes and ongoing
care can help you manage the condition. Lifestyle changes include quitting
smoking, staying active as much as you can, following a healthy diet, getting
plenty of rest, and avoiding situations that can make your symptoms worse.
Follow your treatment plan as your doctor
advises. Take your medicines as prescribed, make any changes in your diet or
exercise that your doctor recommends, keep all of your appointments with your
doctor, and enroll in pulmonary rehab.
Links to Other Information About Idiopathic
Pulmonary Fibrosis
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