Cushing's Syndrome

Overview

What is Cushing’s syndrome (Hypercortisolism)?

Cushing’s syndrome is an uncommon syndrome that happens when your body has too much of a hormone called cortisol. It is also called hypercortisolism. Cortisol is a steroid hormone commonly called the “stress hormone.” Your body releases extra cortisol during times of stress, helping out by:

  • Increasing your heart rate.
  • Increasing your blood pressure.
  • Managing your blood glucose.
  • Managing your respiration.
  • Increasing your muscle tension.

Cortisol also helps by shutting down, temporarily, systems that your body doesn’t need during times of increased stress, such as digestion and reproduction.

Cortisol is essential to:

  • Maintaining blood pressure.
  • Regulating blood sugar.
  • Reducing inflammation.
  • Forming memories.
  • Managing respiration.
  • Balancing salt in your body.
  • Transforming your food into energy.

The adrenal glands (two small glands on top of your kidneys), pituitary gland (in your brain), and the hypothalamus (the part of your brain above the pituitary gland) control cortisol levels.

The cortisol levels found in Cushing’s syndrome are typically caused by a medication or a tumor.

Who is affected by Cushing’s syndrome (Hypercortisolism)?

Children, teenagers and adults, but mostly those aged 25 to 50, are the people most commonly affected by Cushing’s syndrome. Those who take cortisol medication (for example, to treat asthma and rheumatoid arthritis) are especially vulnerable. 70% of people with Cushing’s syndrome are women and 30% are men.

How common is Cushing’s syndrome?

Cushing’s syndrome is rare. 40 to 70 people out of one million are affected each year.

What’s the difference between Cushing’s syndrome (Hypercortisolism) and Cushing’s disease?

Cushing’s disease is a type of Cushing’s syndrome. Cushing’s disease is caused by a benign tumor located in the pituitary gland that secretes too much ACTH (adrenocorticotropic hormone), which in turn increases cortisol.

Out of patients with Cushing’s syndrome, Cushing’s disease accounts for more than 70% of cases in adults and about 60% to 70% of cases in children and adolescents.

What healthcare providers treat Cushing’s syndrome?

Your healthcare provider might consult a specialist called an endocrinologist for evaluation and treatment.

Is Cushing’s syndrome (Hypercortisolism) fatal?

Cushing’s syndrome can possibly be fatal if you don’t get treatment. Without treatment, Cushing’s syndrome can cause health problems including:

  • Infections.
  • Blood clots, especially in the lungs and legs.
  • Depression.
  • Heart attack.
  • Weight gain.
  • Memory problems or difficulty concentrating.
  • High blood pressure or high cholesterol.
  • Broken bones.
  • Type 2 diabetes.

If left untreated, Cushing’s syndrome can also result in death.

Symptoms and Causes

What causes Cushing’s syndrome (Hypercortisolism)?

Too much cortisol causes Cushing’s syndrome. There may be many underlying causes of high cortisol levels, including:

  • Use of glucocorticoid medications. Glucocorticoid medications (for example, prednisone) are used to treat many autoimmune diseases, for example chronic asthma, rheumatoid arthritis, lupus, sarcoidosis and many other diseases that result in chronic inflammation. Chronic treatment with these medications causes “iatrogenic” Cushing’s syndrome. Iatrogenic refers to something that is caused by a medical treatment.
  • Pituitary tumors. Pituitary tumors that make too much ACTH (the hormone that tells the adrenal glands to make cortisol) cause eight out of 10 cases of Cushing's syndrome (excluding the cases of iatrogenic Cushing’s syndrome). This is called Cushing’s disease.
  • Adrenal cortical tumors. A tumor on the adrenal gland itself can make too much cortisol. These are usually benign.
  • Lung, pancreas, thyroid and thymus tumors. Tumors that develop outside of the pituitary gland can produce ACTH and this is called ectopic ACTH syndrome. These types of tumors are typically malignant. The most common type of these tumors is small cell lung cancer.

Is Cushing’s syndrome genetic?

Generally speaking, no, most cases are not genetic.

What are the symptoms of Cushing’s syndrome (Hypercortisolism)?

Cushing’s syndrome has some unique symptoms as well as some that could point towards a variety of other syndromes. Not everyone has the same symptoms. Possible symptoms include:

Rapid weight gain in the face (sometimes called “moon face”), abdomen, the back of the neck (sometimes called “buffalo hump”) and chest.

  • A red, round face.
  • Wounds that heal poorly.
  • High blood pressure (hypertension).
  • Excessive hair growth on the face, neck, chest, abdomen, breasts and thighs, or balding.
  • Diabetes.
  • Purple stretch marks over the abdomen.
  • Easy bruising on the arms and legs.
  • General weakness and tiredness (fatigue).
  • Blurry vision and dizziness.
  • Weak muscles and thinner arms and legs.
  • Libido changes (sex drive) and erectile dysfunction.
  • Stunted growth in children.

How long does Cushing’s syndrome (Hypercortisolism) last?

How long Cushing’s syndrome lasts depends on how you respond to treatment. Professionals note that people with Cushing’s syndrome typically recover after several weeks.

Does Cushing’s syndrome (Hypercortisolism) cause shortness of breath?

No. Shortness of breath is not a symptom of Cushing’s syndrome.

Does Cushing’s syndrome (Hypercortisolism) cause osteoporosis?

Yes. Cushing’s syndrome can weaken bones and that can lead to fractures, especially if your Cushing’s syndrome is long-standing

Does Cushing’s syndrome cause hypokalemia?

Hypokalemia may result from Cushing’s syndrome.

Diagnosis and Tests

How is Cushing’s syndrome (Hypercortisolism) diagnosed?

When your healthcare provider suspects Cushing’s syndrome, he or she will look at your medical history, perform a physical examination and then conduct some laboratory tests.

Cushing’s syndrome can sometimes be difficult to diagnose. If someone reports fatigue and weight gain, that doesn’t point straight at Cushing’s syndrome. It’s also sometimes mistaken for polycystic ovary syndrome or metabolic syndrome. Your healthcare provider will have to go through a process of elimination to rule out other conditions.

What tests will be done to diagnose Cushing’s syndrome?

Your healthcare provider will likely order several tests including:

  • 24-hour urinary cortisol test: This test measures the amount of cortisol in micrograms in your urine. Your healthcare provider will have you collect your urine over a period of 24 hours. 50-100 micrograms per day is a lot and could mean Cushing’s syndrome.
  • Midnight salivary cortisol test: Cortisol levels are known to be very low in the morning, and also low late at night. This test checks cortisol levels between 11:00pm and 12:00am. If you have Cushing’s syndrome, your cortisol levels will be unusually high during that hour.
  • Low-dose dexamethasone suppression test: Dexamethasone is a cortisol-like drug. In this test, one milligram of the drug taken orally at night and then the cortisol levels are measured the following morning. This test determines whether or not the adrenal glands responded to the dexamethasone by suppressing the amount of cortisol they secrete. If you have Cushing’s syndrome, your adrenal glands will not be suppressed, which means your cortisol levels the following morning will remain high. Cortisol levels are known to be very low in the morning. If you don’t have Cushing’s syndrome, you’ll have low levels of cortisol in the morning. If the levels are high, that means you likely have Cushing’s syndrome.
  • Blood test: A blood test will measure the ACTH levels in your blood. An adrenal tumor might be there if the levels are low. If the levels are normal or high, there could be a pituitary or ectopic tumor.
  • High-dose dexamethasone suppression test: This test is like the low-dose dexamethasone suppression test, but the dosage is eight milligrams instead of one. This test is typically performed after the low dose test shows high levels of cortisol in the morning (i.e. failure to suppress), and when blood tests show high ACTH in the blood. This test is used to determine the source of Cushing’s, specifically to differentiate between a pituitary adenoma (Cushing’s disease) and a tumor elsewhere in your body (for example in your lungs).

Once your healthcare provider has confirmed that you have Cushing’s syndrome, the next step is to determine why. Often it is medication or a tumor. If you’re on glucocorticoids, that is probably the cause, and your healthcare provider will likely decrease the dosage. If you’re not on glucocorticoids, that indicates there is a likely a tumor in your adrenal glands, pituitary gland or elsewhere. Your healthcare provider may recommend the following imaging studies to reveal the location of the tumor:

  • Cat scan (CT scan) or MRI abdomen: a CT scan or MRI may be performed to look for a tumor in your adrenal glands. These scans can be done with or without IV contrast. They are very sensitive at identifying adrenal tumors.
  • Magnetic resonance imaging (MRI) pituitary: An MRI will take a picture of your pituitary gland to see if there’s a tumor. In some cases, the MRI will not provide a perfect diagnosis. 50% of those with Cushing’s syndrome will have a “normal” MRI and 10% will have tumors unrelated to the syndrome.
  • Petrosal sinus sampling: This test finds the source of ACTH secretion. ACTH and other pituitary hormones go into the blood stream from the pituitary gland. They do so by going through two veins known as the inferior petrosal sinuses. For the procedure, the healthcare provider will insert a catheter into both veins and look for ACTH in the blood before and after the insertion of a corticotrophin-releasing hormone (CRH) at two, five and 10 minute intervals. This test has a 95% to 98% accuracy.
  • CT scan chest: If a distant tumor is suspected, a CT chest will be performed to evaluate for possible lung cancer.

Management and Treatment

How is Cushing’s syndrome treated?

The type of treatment depends on the underlying cause of the high cortisol levels. If you use glucocorticoids, your healthcare provider will likely lower the dosage or prescribe a non-glucocorticoid medication. If a tumor is causing Cushing’s syndrome, it might need to be killed with radiation or removed surgically. Another option is for your healthcare provider to prescribe a medication such as ketoconazole that will slow down the production of cortisol. You may work with several healthcare providers to treat the tumor and Cushing’s syndrome symptoms.

  • Chemotherapy: Chemotherapy is necessary if a tumor is cancerous and has spread to other parts of your body. Be sure to discuss all side effects with your healthcare provider.
  • Medications: Adding drugs that reduce cortisol or taking away drugs that can cause Cushing’s syndrome.
  • Radiation: Surgery on a pituitary tumor may not be possible. In those cases, you might have to go through a six-week period of radiation. Cortisol levels may take years to return to normal. Medicines that increase cortisol levels are available. Be sure to discuss all side effects with your healthcare provider.
  • Surgery: Surgically removing pituitary tumors, adrenal tumors and ectopic tumors is effective, but you’ll have to be prescribed cortisol medicine. You’ll be able to stop the medications after six to 18 months. Often, after laparoscopic surgery, you’ll be able to leave the hospital within one or two days and feel back to “normal” in about three to five weeks.

If Cushing’s syndrome is properly treated, the disease may go away after two to 18 months. Be sure to stay in contact with your healthcare provider during that period.

Prevention

How can I reduce my risk of or prevent Cushing’s syndrome (Hypercortisolism)?

You always need cortisol in your body. You need it to function. It manages your respiration, turns your food into energy, regulates your blood sugar, helps you cope with stress and more. Cortisol is not your body’s enemy, but too much of it can be. Have your healthcare provider monitor your cortisol levels closely if you’re on glucocorticoids or steroids. Unfortunately, there’s no way to prevent a tumor that causes Cushing’s syndrome.

Outlook / Prognosis

What can I expect if I have Cushing’s syndrome (Hypercortisolism)?

Cushing’s syndrome can be treated. If it isn’t, then it can be fatal. Get your symptoms checked by your healthcare provider as soon as possible.

How long does Cushing’s syndrome last?

Cushing’s syndrome can usually be cured.

Can Cushing’s syndrome get worse?

Cushing’s syndrome can worsen without proper treatment. Be sure to stay in contact with your healthcare provider and report any new or worsening symptoms or any other concerns you may have.

Living With

What is it like living with Cushing’s syndrome (Hypercortisolism)?

Cushing’s syndrome can be difficult to live with, but your quality of life doesn’t have to decrease. There are healthcare providers trained to help you and there are treatments available. With proper procedures and medicine, Cushing’s syndrome can be cured.

You may find yourself dealing with some emotional and social issues as a result of Cushing’s syndrome. Some people may feel embarrassed by the balding, excessive hair and/or the weight gain in the face and back of the neck. “Moon face” and “buffalo hump” may make you hesitate to participate in social situations. With time, those symptoms can be cured. Cushing’s syndrome can also cause depression and other mental illnesses. If need be, consult a therapist for counseling and a psychiatrist for medication to help yourself deal with the emotional impact of Cushing’s syndrome.

When should I see my healthcare provider?

See your healthcare provider if you have the following symptoms of Cushing’s syndrome:

Symptoms that affect females specifically include:

  • New or excessive facial hair.
  • A change in libido (sex drive).
  • A change in the menstrual cycle.

Symptoms that affect males and females include:

  • Rapid weight gain in the face (sometimes called “moon face”), abdomen, the back of the neck (sometimes called “buffalo hump”) and chest.
  • A red, round face.
  • High blood pressure (hypertension).
  • Excessive hair growth on the face, neck, chest, abdomen and thighs.
  • Diabetes.
  • New or excessive acne.
  • Purple stretch marks over the abdomen.
  • Easy bruising on the arms and legs.
  • General weakness and tiredness (fatigue).
  • Blurry vision and dizziness.
  • Wounds that heal poorly.
  • Weak muscles.

What questions should I ask my healthcare provider?

  • What medications can I take to help with my Cushing’s syndrome?
  • Are there any exercises I can do to help my weak muscles?
  • Are there any topical medications that can help with the stretch marks?
  • Do I need to see a specialist about Cushing’s syndrome?
  • Should I stop taking any of my medications?
  • What treatment is best for me?
  • Are there any additional preventative measures I should take?

A note from Cleveland Clinic

Cushing’s is difficult syndrome to endure. It causes weakness, hypertension, fatigue and more. The treatments including chemotherapy and surgery are, at best, uncomfortable. However, they are worth it, because with the right treatment, Cushing’s syndrome can be cured.

Stay in contact with your healthcare provider during every stage. Follow your healthcare provider’s instructions and don’t hesitate to ask them any questions!

Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy

Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy

Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy