Cushing Syndrome

What is Cushing syndrome?

Cushing syndrome is an uncommon condition that happens when your body has too much of a hormone called cortisol. Another word for Cushing syndrome is hypercortisolism. A syndrome is a medical term that refers to a group of signs and symptoms that happen together. You may see some people call this condition Cushing’s syndrome.

Cortisol is a steroid hormone commonly called the “stress hormone.” Your body releases extra cortisol during times of stress. Cortisol helps by:

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

Cortisol also helps by temporarily shutting down 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.

A tumor typically causes the cortisol levels found in Cushing syndrome.

• Endogenous (from within the body): Cushing syndrome happens because of cortisol produced by your body.
• Exogenous (from outside sources): Cushing syndrome happens because of the side effects of certain medications taken to treat other conditions.

Who is affected by Cushing syndrome?

The people most commonly affected by Cushing syndrome are children, teenagers and adults, mostly those ages 25 to 50. People who take cortisol medication (for example, to treat asthma and rheumatoid arthritis) are especially vulnerable. Some 70% of people with Cushing syndrome are women or people assigned female at birth (AFAB) and 30% are men or people assigned male at birth (AMAB).

How common is Cushing syndrome?

Cushing syndrome is rare. It affects 40 to 70 people out of 1 million each year.

What’s the difference between Cushing syndrome and Cushing disease?

Cushing disease is a type of Cushing syndrome. A benign tumor located in the pituitary gland that secretes too much ACTH (adrenocorticotropic hormone) causes Cushing disease. This increases cortisol secretion from the adrenal glands.

Out of all the people who have Cushing syndrome, Cushing 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 syndrome?

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

Is Cushing syndrome fatal?

Cushing syndrome can possibly be fatal if you don’t get treatment. Without treatment, hypercortisolism 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, prediabetes or impaired fasting glucose.

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

What causes Cushing syndrome?

Too much cortisol causes Cushing 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, such as chronic asthma, rheumatoid arthritis, lupus, sarcoidosis and many other diseases that result in chronic inflammation. Chronic treatment with these medications causes “iatrogenic” or exogenous Cushing syndrome. The word “iatrogenic” means that medical treatment has caused something else to happen.
• Pituitary tumors. Pituitary tumors that make too much ACTH (the hormone that tells the adrenal glands to make cortisol) cause 8 out of 10 cases of Cushing syndrome (excluding the cases of iatrogenic Cushing syndrome). The name of this type is Cushing disease.
• Adrenal cortical tumors. A tumor on the adrenal gland itself can make too much cortisol. These are usually benign. However, the tumor can sometimes be an adrenal cortical carcinoma, a very rare adrenal cancer.
• Lung, pancreas, thyroid and thymus tumors. Ectopic ACTH syndrome happens when tumors that develop outside of the pituitary gland produce ACTH. These types of tumors are typically malignant. The most common type is small cell lung cancer.

Is Cushing syndrome genetic?

Generally speaking, no. Most cases of Cushing syndrome aren’t genetic.

What are the symptoms of Cushing syndrome?

Cushing 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 characteristics include:

• Rapid weight gain in the face (sometimes called “moon face”), abdomen, 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.

Other signs and symptoms of Cushing syndrome include:

• 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 syndrome last?

How long Cushing syndrome lasts depends on how you respond to treatment. Many people with hypercortisolism recover after several weeks of treatment.

Does Cushing syndrome cause shortness of breath?

No. Shortness of breath isn’t a characteristic symptom of Cushing syndrome.

Does Cushing syndrome cause osteoporosis?

Yes. Cushing syndrome can weaken bones. That can lead to fractures, especially if you have a long-standing case of Cushing syndrome. Weak bones can cause pain.

Does Cushing syndrome cause hypokalemia?

Hypokalemia is the medical term for having low levels of potassium in your blood. This may happen if you have Cushing syndrome.

How is Cushing syndrome diagnosed?

When your healthcare provider suspects hypercortisolism, there are certain guidelines they may follow. They’ll ask questions, look at your medical history, perform a physical examination and then conduct some laboratory tests. They’ll likely continue to monitor you over time.

Cushing syndrome can sometimes be difficult to diagnose. If you tell your provider you have fatigue and weight gain, they might not immediately think of Cushing syndrome. These types of symptoms are common to many different kinds of diseases.

Cushing syndrome is 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 syndrome?

Your healthcare provider is likely to request some of the following tests:

• 24-hour urinary cortisol test: This test measures the amount of cortisol in micrograms (mcg) in your urine. Your healthcare provider will have you collect your urine (pee) over a period of 24 hours.
• Midnight salivary cortisol test: Typically, cortisol levels are very low late at night. This test checks cortisol levels between 11 p.m. and 12 a.m. If you have Cushing syndrome, your cortisol levels will be unusually high during that hour.
• Low-dose dexamethasone suppression test: Dexamethasone is a cortisol-like drug. For this test, you take one milligram (mg) of the drug by mouth at night and then measure cortisol levels between 8 a.m. and 9 a.m. This blood test determines if the adrenal glands responded to the dexamethasone by suppressing the amount of cortisol they secrete. If you have Cushing syndrome, your cortisol levels will remain high.
• 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 8 milligrams instead of one. A provider will typically perform this test after the low-dose test shows high levels of cortisol in the morning and when blood tests show high ACTH in the blood. This test can determine the source of Cushing syndrome, since it can tell the difference between a pituitary adenoma (Cushing disease) and a tumor elsewhere in your body (such as your lungs).

Once your healthcare provider has confirmed that you have Cushing syndrome, the next step is to determine why. Often it’s medication or a tumor. If you’re on glucocorticoids, that’s probably the cause, and your healthcare provider will likely decrease the dosage. If you’re not on glucocorticoids, that indicates there’s 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: Your provider may perform a CT scan or MRI to look for a tumor in your adrenal glands. The provider can do these scans with or without IV contrast. The tests 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 won’t provide a perfect diagnosis. Fifty percent of those with Cushing syndrome will have a “normal” MRI and 10% will have tumors unrelated to the syndrome.
• Bilateral inferior petrosal sinus sampling (BIPPS): This test finds the source of ACTH secretion. ACTH and other pituitary hormones go into the bloodstream from the pituitary gland. An experienced interventional radiologist will go through two veins known as the inferior petrosal sinuses. This test has a 95% to 98% accuracy rate.
• CT scan chest: If your provider suspects an ectopic tumor, they’ll order a CT chest to look for possible lung cancer.

How is Cushing 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’s causing Cushing syndrome, your provider may suggest surgery or radiation.

Another option is for your healthcare provider to prescribe a medication such as ketoconazole that’ll slow down the production of cortisol. You may work with several healthcare providers to treat the tumor and Cushing syndrome symptoms.

• Chemotherapy: Chemotherapy is necessary if a tumor’s 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 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. 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 adjust to new, lower cortisol levels. During this time, you may need to take cortisol medications in the form of hydrocortisone tablets. You may 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.

If Cushing 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 and after this period.

How can I reduce my risk of or prevent Cushing syndrome?

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 isn’t your body’s enemy, but too much of it can be. However, you can’t live without cortisol.

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 syndrome (hypercortisolism).

What can I expect if I have Cushing syndrome?

Your provider can and should treat Cushing syndrome. If it’s not treated, it can be fatal. Get your symptoms checked by your healthcare provider as soon as possible.

How long does Cushing syndrome last?

There’s usually a cure for Cushing syndrome. Treatment may last for some time, even up to 18 months.

Can Cushing syndrome get worse?

Cushing syndrome can get worse 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.

What’s it like living with Cushing syndrome?

Cushing syndrome can be difficult to live with, but your quality of life doesn’t have to get worse. There are healthcare providers trained to help you and there are treatments available. Generally, these will be able to cure Cushing syndrome and improve symptoms caused by hypercortisolism.

You may find yourself dealing with some emotional and social issues due to Cushing 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, treatment can cure those symptoms. Cushing 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 hypercortisolism.

When should I see my healthcare provider?

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

Symptoms that affect women and people AFAB specifically include:

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

Symptoms that affect everyone 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 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 syndrome?
• Should I stop taking any of my medications?
• What treatment is best for me?
• Are there any additional preventive measures I should take?

A note from Cleveland Clinic

Cushing syndrome can be a difficult syndrome to endure. It causes weakness, hypertension, fatigue and more. The treatments — including surgery, medications, radiation and chemotherapy — may be uncomfortable. However, they’re worth it because, with the right treatment, there’s a cure for Cushing syndrome.

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.