Topic Overview
What is Cushing's syndrome?
Cushing's syndrome is a
rare hormonal problem. It happens when there is too much of the
hormone cortisol in your body.
Normally,
cortisol levels increase through a chain reaction of hormones:
- First, the hypothalamus in the brain makes a hormone called CRH
(corticotropin-releasing hormone).
- Then, CRH tells the pituitary
gland to make another hormone called ACTH
(adrenocorticotropic hormone).
- ACTH then
tells the
adrenal glands to make cortisol. Cortisol affects
almost every area of the body. It is especially important in regulating blood
pressure and
metabolism.
But if your body makes too much cortisol—or if you take
certain medicines that act like cortisol—you may start to have symptoms.
Cushing's syndrome may cause weight gain, skin changes, and fatigue. It can
lead to serious problems, such as
diabetes,
high blood pressure,
depression, and
osteoporosis. If not treated, it can also cause
death.
Another name for Cushing's syndrome is
hypercortisolism.
What causes Cushing's syndrome?
Cushing's syndrome
may be caused by:
-
Steroid medicine
. This medicine
is used to treat
lupus,
asthma,
rheumatoid arthritis, and other diseases that cause
inflammation. It may also be used after an organ transplant. Long-term use of
this medicine is the most common cause of Cushing's syndrome.
-
Tumors in the pituitary gland. This is called Cushing's
disease. It's the second most common cause of Cushing's syndrome. These tumors
are not cancer.
-
Tumors in the adrenal glands, or in the lung or pancreas. Sometimes these tumors are cancer.
What are the symptoms?
Weight gain—especially
around the waist—is the most common symptom.
Cortisol affects
almost all body systems, so it can cause many other symptoms. These other
symptoms often appear slowly over time and may include:
- Weak muscles.
- Changes in the
skin, such as bruising,
acne, and dark purple-red stretch marks on the
belly.
- Changes in mood. You may feel irritable, anxious, or
depressed.
- Extra fat on the back of the neck and upper
back.
- Backaches.
- Loss of muscle
tone.
- Irregular
menstrual periods.
- Hair growth that is not
normal (such as too much facial hair in women).
- High blood pressure and
high blood sugar levels.
Sometimes alcoholism, depression,
panic attacks, obesity, or other problems can cause
symptoms like these. Some treatments for
HIV can also cause similar symptoms. This is called
pseudo-Cushing's syndrome. In these cases, symptoms tend to stop as soon as the
problems are treated.
How is Cushing's syndrome diagnosed?
Your doctor
will use your medical history, a physical exam, and lab tests to see if you
have Cushing's syndrome. During the physical exam, he or she will look for
signs of the problem. The medical history includes questions about your
symptoms, what medicines you take, and—if you are a woman—whether your periods
are regular.
If your doctor thinks you may have Cushing's
syndrome, you will have lab tests to check your cortisol levels. These tests
can measure cortisol in your blood, urine, or saliva. More tests may be needed
to find the cause of high cortisol levels.
How is it treated?
Cushing's syndrome can often be
cured. But it can lead to serious health problems, including death. So it's
important to start treatment right away.
If steroid medicine is
causing Cushing's syndrome, your doctor will help you lower your dose or
gradually stop taking it. It may take a while for the symptoms to go away.
It's very dangerous to stop taking steroid medicine on your own.
Your doctor will help you change your medicine or lower your dose
slowly.
If a tumor is causing Cushing's syndrome, you will need
surgery to remove it. If surgery doesn't work, radiation or medicines may be
used.
When you have Cushing's syndrome, it's very important to
control your weight and keep your bones and muscles strong. This will help
prevent diabetes, bone loss, and high blood pressure. Eating healthy foods and
staying active can help you do this.
It's also important to have
regular checkups to look for other problems such as diabetes, high blood
pressure, and osteoporosis.
Frequently Asked Questions
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Learning about Cushing's syndrome:
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Being diagnosed:
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Getting treatment:
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Living with Cushing's syndrome:
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Symptoms
The signs and symptoms of
Cushing's syndrome vary and most often develop
gradually. You may have:
-
Weight gain. Symptoms may include a round or puffy
face (moon face), increased fat around the neck and upper part of the back
(buffalo hump), or an enlarged waistline. Weight gain is the most common symptom
of Cushing's syndrome.
-
Skin changes. These include thin, fragile skin that
bruises easily; slow-healing wounds; wide, purplish stretch marks on the body
(striae);
acne; or a ruddy complexion.
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Changes in mental state. Irritability, anxiety,
inability to sleep (insomnia), or feelings of sadness or
depression may develop.
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Muscle and bone weakness. Symptoms may include
backache; broken bones, especially the ribs and spine (caused by
osteoporosis); or loss of muscle tone and strength.
Weakness of the muscles in the arms and legs may make it difficult to rise from
a chair or climb stairs.
-
Sex hormone changes. Menstrual irregularity, facial hair growth in women,
erection problems (erectile dysfunction), or changes in
sex drive may occur.
-
High blood pressure and diabetes. High blood pressure (hypertension) and
type 2 diabetes may not be diagnosed until you have a
checkup.
The eye conditions
glaucoma and
cataracts also may occur in Cushing's syndrome. In
Cushing's disease (tumors on the
pituitary gland), your field of vision can be
affected. You may have loss of side, or peripheral, vision.
If a
cancerous tumor on the lung is the cause, you may have rapid loss of appetite
(anorexia) and weight loss. You also may have dark
spots on the skin (hyperpigmentation).
Not everyone who has the
classic signs and symptoms of Cushing's syndrome has the disorder. Also, not
everyone who has Cushing's syndrome has the classic changes in physical
appearance. This is often true in people who are very physically active.
Exams and Tests
Cushing's syndrome can be difficult to diagnose because a variety of factors—from
the time of day to a temporary illness—can lead to a higher-than-average
cortisol level in people who do not have Cushing's
syndrome. It also can be hard to pinpoint the cause. This is important,
because treatment depends on the cause. You may need to see an
endocrinologist (a doctor who specializes in hormone
disorders) to diagnose or treat Cushing's syndrome.
To diagnose
Cushing's syndrome, your doctor will ask about your
medicines, symptoms, history of cancer or smoking, and any changes to your menstrual periods (if you are a woman). Your doctor will also do a physical exam. He or she will check for high blood pressure and look at your skin for color changes, bruising, stretch marks, and changes in thickness. Your doctor will also check for changes in your weight and for any signs of cancer. Your doctor can usually find out
from these exams whether
corticosteroid medicine is causing the condition. If
it is, other tests usually are not done and your doctor will consider changing
your medicine.
If you are not taking steroid medicine or your
doctor believes something other than steroid medicine is causing Cushing's
syndrome, you will have laboratory tests to check your level of cortisol. These
tests include:
- Tests to check for
cortisol in blood or
cortisol in urine.
- An
overnight dexamethasone suppression test using a steroid medicine (often done to reconfirm a cortisol test). This test is most
commonly done on a sample of blood, but a more extensive form of the test may
involve both blood and urine samples.
- A test to measure cortisol in the saliva at midnight may
be done.
If the above tests show you have Cushing's syndrome, the
following blood tests can help your doctor find out the amount of
adrenocorticotropic hormone (ACTH) in your body and
whether you have a tumor on the
adrenal glands, the
pituitary gland, or an organ:
If the first tests show that too much ACTH is causing
Cushing's syndrome, other tests may be needed to find out its source. These
include:
- Inferior petrosal sinus sampling (IPSS) to find
out whether too much ACTH is being released from the pituitary gland. During
IPSS, a small tube (catheter) is used to collect samples from blood vessels
near the brain. If these blood samples show high levels of ACTH, it usually
means that the pituitary gland is the source of excess ACTH. The IPSS is
often used with the CRH stimulation test.
-
Computed tomography
(CT scan) and
magnetic resonance imaging (MRI) of the chest or
pituitary gland, which may help locate a tumor producing ACTH. But sometimes
the tumor may be too small to detect with a CT scan or an MRI. A CT and MRI of
the adrenal gland may also be used to locate adrenal tumors.
Blood test results showing changes in body chemistry also
may point to Cushing's syndrome. These include:
- An increase in the number of white blood cells
(leukocytosis).
- A low potassium level
(hypokalemia).
- High blood sugar
(hyperglycemia).
- Increased levels of
cholesterol (hypercholesterolemia) and high blood fats
(hypertriglyceridemia).
- A decrease in the time it takes for blood
to clot.
- A high
testosterone level.
Treatment Overview
Treatment for
Cushing's syndrome depends on the cause.
For Cushing's syndrome caused by long-term corticosteroid medicine use
If
corticosteroid medicine is the cause of Cushing's syndrome, your doctor will help you lower your dose or
gradually stop taking it. Never stop taking
corticosteroid medicine on your own, because it might
lead to a life-threatening adrenal crisis. When you take steroids, your
adrenal glands stop making
cortisol. If you suddenly stop taking your medicine,
your adrenal glands may not be able to start making cortisol quickly enough.
This can lead to an adrenal crisis and a severe drop in blood pressure. To
avoid this, your doctor will want to gradually reduce and then stop your
medicine.
Your doctor may change your corticosteroid medicine from
a longer-acting steroid (such as prednisone) to a shorter-acting one (such as
hydrocortisone). Sometimes corticosteroid medicines can be taken every other
day. Either way, the body's normal production of cortisol returns
gradually.
If you must continue taking corticosteroid medicine to
control another condition, the dosage can sometimes be lowered to reduce
symptoms and the risk of complications.
If your doctor and you are
trying to reduce the dosage of your medicine and you become ill, contact your
doctor immediately.
If reducing the dosage does not make Cushing's
syndrome go away, your doctor will perform more tests to look for another cause
of your condition.
For Cushing's syndrome caused by pituitary tumors (Cushing's disease)
If you are well enough to have surgery, surgical removal
of the pituitary tumor offers the best chance for recovery. The surgery (transsphenoidal adenomectomy) requires great skill and
should be performed at a major medical center where teams of doctors specialize
in pituitary surgery.
Gamma knife radiosurgery
has recently been introduced in the United States. In this technique, many
small beams of
radiation are focused on the tumor to shrink and
destroy it. It does not involve a surgical incision (there is no "knife"
involved), and there is minimal damage to surrounding tissue. It can be done as
an outpatient and with
local anesthesia. Few centers in the United States
have gamma knife facilities.
Medicine therapies may be tried if
surgery is not possible or has failed.
For Cushing's syndrome caused by adrenal tumors
Doctors almost always recommend surgery to remove benign adrenal tumors
that are producing hormones. If a tumor is cancerous, the affected adrenal
gland is removed. Although chemotherapy is usually advised, there is no proven
long-term treatment for adrenal cancer. On rare occasions, both adrenal glands
must be removed. In this case, you would take daily long-term hormone
replacement.
Surgery is usually successful if the tumor is not
cancerous. If the tumor is cancerous, success depends on how much the cancer has spread.
If surgery is not possible, medicine therapy may be tried to reduce the
tumor's production of cortisol. This includes using ketoconazole (Nizoral),
mitotane (Lysodren), and aminoglutethimide (Cytadren). These medicines also are
sometimes used before surgery in people who have severe Cushing's syndrome.
Pregnant women who have Cushing's disease can take aminoglutethimide.
For Cushing's syndrome caused by tumors of the lungs and elsewhere
To successfully treat Cushing's syndrome caused by a
noncancerous (benign) or cancerous tumor, the tumor tissue that is secreting
adrenocorticotropic hormone (ACTH) must be destroyed
or removed. Surgery, chemotherapy, radiation therapy, immunotherapy, or a
combination of treatments may be recommended to treat the tumor.
If left untreated for a long time, Cushing's syndrome
may cause serious problems, including complications from
high blood pressure (such as a
heart attack or
stroke),
osteoporosis, or
type 2 diabetes. The condition also can cause death. Because
of these risks, treatment usually begins as soon as possible.
Home Treatment
Home treatment for
Cushing's syndrome consists of lifestyle changes to
prevent weight gain, to strengthen muscles and bones, and to prevent
complications.
- Eat a low-calorie, nutritious diet high in
protein and calcium. This can help prevent muscle and bone loss caused by the
high cortisol levels in your body. Take calcium and vitamin D supplements to
decrease bone loss. Ask your doctor whether you need medicine to help slow bone
loss. For more information, see the topic
Healthy Eating.
- Limit
salt (sodium) in your diet. This is especially
important if you have
high blood pressure, a complication of Cushing's
syndrome.
Healthy Eating: Eating Less Sodium
- Get regular exercise to help maintain muscles and bone
mass and prevent weight gain. To maintain muscle and bone mass, try
weight-bearing exercises such as push-ups, sit-ups, or lifting weights. To
prevent weight gain, try aerobic exercise to increase your heart rate. Examples
of aerobic exercise include fast walking, jogging, cycling, and swimming.
Consult your doctor before you start any exercise program. For more
information, see the topic
Fitness.
- Avoid possible falls by removing
loose rugs and other hazards from your home. Falling may lead to broken bones
and other injuries.
- Pay close attention to all wounds. Too much
cortisol slows wound healing. Clean all wounds immediately with antibacterial
soap and use antibiotic ointment and dressings to prevent
infection.
- Seek counseling if you need help dealing with changes in
your body image.
- Get regular eye exams to check for
glaucoma and
cataracts.
- See your doctor regularly to
help diagnose and treat
diabetes, high blood pressure, and other potential
complications.
If you do not have Cushing's syndrome but are taking
corticosteroid medicines, talk to your doctor about
whether you are at risk for the syndrome. Your doctor may reduce
your dosage, treat your condition with a different medicine, or prescribe a
shorter-acting corticosteroid medicine.
Other Places To Get Help
Organizations
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Cushing's Support and Research Foundation,
Inc.
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| 65 East India Row |
| Suite 22-B |
| Boston, MA 02110 |
| Phone: |
(617) 723-3674 |
| Fax: |
(617) 723-3674 |
| Email: |
cushinfo@csrf.net |
| Web Address: |
http://csrf.net |
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The Cushing's Support and Research Foundation offers consumer
pamphlets and other information. The organization also publishes a
newsletter.
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Hormone Health Network
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| 8401 Connecticut Avenue |
| Suite 900 |
| Chevy Chase, MD 20815-5817 |
| Phone: |
1-800-HORMONE (1-800-467-6663) |
| Web Address: |
www.hormone.org |
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The Hormone Health Network is a nonprofit organization
started by the Endocrine Society. The organization promotes the prevention,
treatment, and cure of hormone-related conditions through public outreach and
education.
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National Adrenal Disease Foundation
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| 505 Northern Boulevard |
| Suite 200 |
| Great Neck, NY 11021 |
| Phone: |
(516) 487-4992 |
| Email: |
nadfmail@aol.com |
| Web Address: |
www.nadf.us |
| |
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The National Adrenal Diseases Foundation is a consumer-based
organization providing information and support for people with adrenal-related
diseases.
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National Endocrine and Metabolic Diseases Information
Service
|
| 6 Information Way |
| Bethesda, MD 20892–3569 |
| Phone: |
1-888-828-0904 |
| Fax: |
(703) 738-4929 |
| Email: |
endoandmeta@info.niddk.nih.gov |
| Web Address: |
http://endocrine.niddk.nih.gov |
| |
|
The National Endocrine and Metabolic Diseases Information Service
is a service of the National Institute of Diabetes and Digestive and Kidney
Diseases, National Institutes of Health. This Web site offers consumer
information on the cause, treatment, and effects of endocrine and metabolic diseases.
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Pituitary Network Association (PNA)
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| P.O. Box 1958 |
| Thousand Oaks, CA 91358 |
| Phone: |
(805) 499-9973 |
| Fax: |
(805) 480-0633 |
| Email: |
PNA@pituitary.org |
| Web Address: |
www.pituitary.org |
| |
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The Pituitary Network Association is a nonprofit
organization that provides support for people who have pituitary tumors and
disorders.
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References
Other Works Consulted
- Almeida MQ, Stratakis CA (2011). Cushing’s syndrome. In ET Bope et al., eds., Conn’s Current Therapy 2011, pp. 653–659. Philadelphia: Saunders.
- Carroll TB, et al. (2011). Glucocorticoids and adrenal androgens. In DG Gardner, D Shoback, eds., Greenspan’s Basic and Clinical Endocrinology, 9th ed., pp. 285–327. New York: McGraw-Hill.
- Loriaux DL (2009). Adrenal. In EG Nabel, ed., ACP Medicine, section 3, chap. 4. Hamilton, ON: BC Decker.
- Nieman L, et al. (2008). The diagnosis of Cushing's syndrome: An Endocrine Society clinical practice guideline. Journal of Clinical Endocrinology and Metabolism, 93(5): 1526-1540.
- Stewart PM, Krone NP (2011). The adrenal cortex. In S Melmed et al., eds., Williams Textbook of Endocrinology, 12th ed., pp. 479–544. Philadelphia: Saunders.
Credits
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By
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Healthwise Staff |
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Primary Medical Reviewer
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E. Gregory Thompson, MD - Internal Medicine |
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Specialist Medical Reviewer
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David C.W. Lau, MD, PhD, FRCPC - Endocrinology |
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Last Revised
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January 10, 2012 |