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 lead to
longer and better quality lives for people with IPF. 

What causes idiopathic pulmonary fibrosis? 

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 alveoli (air sac walls)
into the bloodstream. 

Doctors think the following things may increase your chances of developing
IPF:

Chemotherapy or cancer medicines, such as methotrexate, cyclophosphamide,
and bleomycin

Your genes may also play a role in the development of IPF.  Some families
have at least two members with IPF. 

Scientists have found that 9 out of 10 people with IPF also have gastroesophageal
reflux disease (GERD).  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. 

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 do appear, they are likely to become worse
over time. 

The most common signs and symptoms are:

Shortness of breath.  This is usually the main symptom.  At first, you
may be short of breath only during exercise.  Over time, you are likely
to feel breathless even at rest. 

A dry, hacking cough that doesn't get better when you treat it with
over-the-counter cough medicines.  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

Enlargement of the ends of the fingers or toes, which is called clubbing

IPF may lead to other medical conditions, including: collapsed lung, lung
infections, blood clots in the lungs, and lung cancer. 

How is idiopathic pulmonary fibrosis diagnosed? 

Idiopathic pulmonary fibrosis (IPF) produces 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 (a doctor who specializes
in lung problems), as soon as you have symptoms is important. 

To diagnose IPF, your doctor will ask about your medical history, perform
a physical exam, and do several tests to rule out other causes of your symptoms
and learn how badly your lungs are damaged. 

Medical history

A complete medical history is important.  Your doctor will 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 that can trigger an immune
system response

Your family's medical history

How long you've had symptoms

Diagnostic tests

There is no single test for diagnosing IPF.  Your doctor will probably conduct
several different tests, including the following. 

High-resolution computerized tomography (HRCT) scan is an X-ray that provides
sharper and more detailed images than a regular chest X-ray.  It 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. 

Your doctor will do a lung function test called spirometry
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 there
is a lot of scarring in your lungs, you will not be able to move a normal
amount of air out of them. 

The doctor takes a small amount of blood from an artery
in your arm, usually in your wrist.  It's 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 can also
be tested to see whether you have an infection that may be causing your
symptoms. 

Skin test for tuberculosis

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 used 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.  Electrodes
attached to your chest and an EKG
(electrocardiogram) machine show your heart
rate, a blood pressure cuff monitors your blood pressure, and a pulse
oximeter attached to a finger or ear lobe shows how much oxygen is in your
blood. 

Sometimes doctors place a catheter in an artery in one of your arms to draw
samples of your blood that will provide a more accurate measure of the oxygen
and carbon dioxide in it.  They may also ask you to breathe into a tube that
separates the air you breathe out from room air and provides a precise measure
of the amount of oxygen you've taken in and carbon dioxide you're breathing
out. 

Lung biopsy

Looking at samples of tissue from several places in your lungs under a microscope
is the best way for your doctor to diagnose IPF.  A lung biopsy
can help your doctor rule out other causes of your condition, such as sarcoidosis,
cancer, or infection.  It can also show your doctor how far your condition
has advanced.  Doctors use several different procedures to obtain samples
of your lung tissue:

Bronchoscopy.  Your doctor inserts
a flexible, rubber tube with a tiny light and camera on the end through
your nose or mouth into your lungs to look at your airways.  He or she
then inserts a forceps through the tube to collect tissue to examine. 
This procedure is usually done under local anesthesia
on an outpatient basis. 

Bronchoalveolar lavage.  During bronchoscopy, your doctor may inject
a small amount of salt water (saline) through the bronchoscope 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. 

Video-assisted thoracoscopy.  This is the procedure that doctors use
most to obtain lung tissue.  Your doctor inserts a small, lighted tube
with a camera (endoscope)
into your chest through small incisions 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 the hospital, under
general anesthesia. 

Thoracotomy.  Your doctor removes a few small pieces of lung tissue
through an incision in the chest wall between your ribs.  Thoracotomy
is done in the hospital under general anesthesia. 

How is idiopathic pulmonary fibrosis treated? 

The goals of treating idiopathic pulmonary fibrosis (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 is usually 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.  Sometimes 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 difficulty sleeping at night (insomnia), weight gain,
acne, and irritability.  Using prednisone for
a long time also can lead to other conditions, including:

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 – a doctor who specializes
in the diagnosis and treatment of the adrenal glands. 

Prednisone also can cause conditions such as diabetes and glaucoma to get
worse. 

Other medicines

Many doctors prescribe a second medicine with prednisone. 

Azathioprine (ImuranŽ) is a medicine
that 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:

Cyclophosphamide (CytoxanŽ) 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 receive
it for 3 to 5 days through a needle that is inserted in 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 order blood
tests before, during, and after your treatment to see how your blood
cells are affected by the drug.  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 with IPF include the following:

Influenza and pneumonia vaccinations
may help prevent infection and keep you healthy. 

Cough medicines or oral codeine may relieve coughing. 

Vitamin D, calcium, and a bone-building drug may help prevent bone
loss if you are taking prednisone or another corticosteroid. 

Anti-reflux therapy may help control gastroesophageal reflux disease. 

New medicines being studied

Researchers are studying new treatments for IPF.  Some are looking at medicines
that may reduce inflammation and/or prevent or reduce scarring in IPF.  These
include medicines that are used to treat other conditions. 

N-acetylcysteine. 
N-acetylcysteine is an antioxidant that's
used mostly to thin mucus in patients with other lung conditions.  A recent
study showed that adding it to prednisone and azathioprine helped prevent
further damage to the lungs of people with IPF. 

Interferon
gamma-1b.  Interferon gamma-1b is a manmade version of a substance that
your body normally produces to help fight infections.  The drug is usually
injected under your skin three times a week.  The most common side effects
include fever, headache, muscle soreness,
fatigue, and chills. 

Etanercept.  Etanercept is a medicine
that's used to reduce signs and symptoms of active arthritis or rheumatoid
arthritis, such as joint swelling, pain, tiredness, and morning stiffness. 
Etanercept is also used to slow the progress of arthritis.  The most common
side effects are chills, cough, fever, sneezing, and sore throat. 

Bosentan.  Bosentan is a medicine developed to reduce high blood
pressure in the blood vessels of the lungs (pulmonary arterial hypertension). 
You usually take this drug by mouth once or twice a day.  The most common
side effects include headaches, flushing, leg swelling, dizziness and fatigue,
and liver problems.  If you take this medicine, your doctor should test your
blood often to monitor how your liver is working. 

Imatinib. 
Imatinib is a medicine developed to treat a type of leukemia.  The drug is
usually given by mouth once a day.  The most common side effects include
fluid retention (swelling), nausea, vomiting, diarrhea, muscle cramping,
and liver problems.  If you take this medicine, your doctor should do frequent
blood tests. 

Pirfenidone.  Pirfenidone is a medicine that
may decrease scarring.  The drug is usually taken by mouth three times a
day.  The most common side effects include a rash and sun sensitivity, nausea,
vomiting, loss of appetite, drowsiness, and fatigue. 

Colchicine. 
Colchicine is a medicine that has been used to prevent or treat attacks
of gout.  It may slow scarring in IPF.  You usually take it by mouth once
or twice a day.  The side effects are generally less serious than those from
prednisone.  They may include nausea, vomiting, stomach pain, and diarrhea. 

Methotrexate.  Methotrexate is an immune system suppressant
that's used to treat some cancers and autoimmune diseases.  Most people take
it once a week by mouth or injection.  People with IPF usually take it for
at least 4 to 6 months to see if it works.  You may have side effects, especially
if you take it at high doses. 

The most serious possible side effect is liver damage.  Your doctor should
monitor how your liver is working every month that you're on this drug. 
If you take methotrexate for more than 2 years, you may want to have a liver
biopsy to make sure your liver hasn't been damaged and that you can continue
to take the medicine. 

Other side effects may include nausea, mouth sores, skin rash, a decrease
in infection-fighting white blood cells (requiring regular blood tests to
check white cell levels), and an allergic reaction in the lungs that goes
away when you stop taking the drug (this is very rare). 

Your doctor should also monitor your white blood cells each month while
you're on methotrexate.  You can often reduce your chances of having bad
side effects from methotrexate by taking folic
acid. 

Penicillamine.  Penicillamine is used to treat
rheumatoid arthritis and to prevent kidney stones.  It's available in pill
or capsule form.  Several small studies have shown that penicillamine can
improve lung function in some IPF patients, but more research is needed. 

Penicillamine can cause serious side effects.  The most common include nausea,
vomiting, diarrhea, dyspepsia, and anorexia. 

Cyclosporine. 
Cyclosporine is another immune system suppressant.  It's used mostly to prevent
rejection of kidney, liver, and heart transplants.  Some doctors think it
may help people with IPF reduce their dose of corticosteroids while waiting
for a lung transplant.  More research
is needed. 

Oxygen therapy

When the amount of oxygen in your blood gets low, you may need oxygen therapy
to help reduce your shortness of breath and let you be more active.  Oxygen
is usually 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 rehabilitation is now the standard of care for people with ongoing
lung disease.  It 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 transplantation

Early referral for surgery to replace one of your lungs with a healthy lung
from a human donor is usually recommended if you:

Are younger than 65

Have no other medical problems

Are not being helped by medicines

Single lung transplantation 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 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 is no cure for idiopathic pulmonary fibrosis (IPF) yet.  Understanding
that your symptoms may get worse over time is important.  As your symptoms
get worse, you may not be able to do many of the things that you did before
you had IPF. 

If you're still smoking, the most important thing you can do is stop.  Ask
your doctor or nurse about smoking cessation programs and products that
can help.  Avoid secondhand smoke as well.  Ask family members and friends
not to smoke in front of you or in your home, car, or workplace. 

Ongoing medical care is important.  Treatment by a pulmonologist who specializes
in IPF is usually recommended.  These specialists are usually located at
major medical centers. 

Treatment may relieve your symptoms and even slow or stop the fibrosis (scarring). 
Following your treatment plan is very important.  You should:

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

Being as active as you can is important to both your physical and mental
health.  It 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 you do this. 

As your condition advances, use a wheelchair or motorized scooter, or stay
busy with activities that aren't physical in nature. 

As your condition worsens, you may need oxygen full time.  Some people with
IPF carry portable oxygen when they go out. 

You should also follow a healthy diet.  Eating smaller, more frequent meals
may relieve stomach fullness, which can make it difficult 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 disease like IPF. 

Maintain a positive attitude.  Practicing relaxation techniques may help
you do this.  It may also help you avoid excessive oxygen intake caused by
tension or overworked muscles. 

Join a support group to help you adjust to living with IPF.  Talk to others
who have the same symptoms so you can see how they're coping.  To find a
local support group, check your telephone directory or contact the Coalition
for Pulmonary Fibrosis or the Pulmonary Fibrosis Foundation. 

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.