What is Cushing Disease?

Cushing disease, also called Cushing’s disease, Cushing syndrome or hypercortisolism, is a rare hormone disorder caused by an excess of cortisol in the body.

This endocrine disorder is most common in women, ages 20 to 50. Cushing syndrome affects about 13 million people annually, and an estimated 70% of those cases are women (AANS). 

The key to treating Cushing disease is catching it in its earliest stages, before symptoms appear, or worsen.

What is cortisol?

What is cortisol, and why is it so important that our cortisol levels are in check? 

Cortisol is the body’s primary stress hormone, and it plays the critical roles of increasing blood sugar, regulating the body’s stress response, reducing inflammation, and regulating metabolism.

The adrenal glands on the kidneys are responsible for producing cortisol. When cortisol is too low (also known as Addison’s disease), a person may feel weak, fatigued, and have low blood pressure. When cortisol is too high (Cushing disease), a person may experience rapid weight gain, high blood pressure, and bone loss.

Cortisol levels in the body will vary based on the time of day. When cortisol is “normal” or healthy, test results may show anywhere from:

• 10 to 20 micrograms per deciliter (mcg/dL) – between 6 and 8 a.m.
• 3 to 10 mcg/dL after 4 p.m.

Cortisol measurements outside of these ranges are considered abnormally high, or abnormally low. Both Addison’s disease and Cushing’s disease have effective medication treatment options.

What are the signs and symptoms of Cushing disease?

• Weight gain: One of the main signs of Cushing disease is weight gain. Fat deposits tend to form around the face, above the collar bone, and on the back of the neck. Weight gain often affects the chest and abdomen area, while the arms and legs remain thinner. 

• Skin changes: Hypercortisolism can cause a number of skin changes, including easy bruising, thinning skin, purple stretch marks (also called striae) around the abdomen, reddening of the face, balding, and excessive hair growth (hirsutism) around the face, chest, and abdomen.

• Muscle changes: A person with high cortisol levels may feel weak and fatigued often. Cushing disease may even lead to muscular dystrophy if left untreated. Muscle weakness can unfortunately persist even after the syndrome is treated, so it’s essential to catch this disease as soon as possible.

• Mood changes: Cushing’s disease is known to cause severe depression, irritability, anxiety, and mood swings, as well as decreased fertility and/or libido. 

• Health changes: Over time, Cushing disease may lead to type 2 diabetes, heart disease, cause menstrual disorders in women, vertigo, brittle bones, and sleep disorders and/or sleep apnea. (Do you know how to prevent heart disease?)

Cortisol plays a role in all of our critical functions, so having abnormally high cortisol levels can have a significant impact on every aspect of life – from your mood, to your strength, sleep quality, and overall health.

Causes of Cushing Disease

Cushing disease may be caused by an overuse of corticosteroids, such as prednisone, or may be due to an underlying condition, such as an adrenal tumor, lung tumor, or pituitary tumor.

Chronic stress and poor stress management can cause Cushing disease, as cortisol is the hormone we produce when we’re stressed. Similarly, panic disorders that cause your cortisol to rise regularly, can also cause Cushing disease.

While genetics are not a leading cause of Cushing’s disease, it is possible that a person is genetically predisposed to tumors on the adrenal glands.

Risk Factors of Cushing Disease

Women, ages 20 to 50, are at the highest risk of developing Cushing disease. Other risk factors include having diabetes, high blood pressure (hypertension), kidney stones, being obese, and living with pituitary or adrenal tumors.

It’s important to visit your doctor regularly, to ensure that you don’t have undiagnosed diabetes or another underlying condition. (What are the 3 most common symptoms of undiagnosed diabetes?)

How is Cushing’s disease diagnosed?

If you suspect that you may have Cushing disease, schedule an appointment with one of our experienced endocrinologists in Orlando to ensure that you get the proper testing done. 

Your doctor may perform a physical exam to assess for signs of Cushing’s disease, discuss your current medications and health history, and order a series of lab tests. The right lab test(s) for you will depend on your specific case. These may include any of the following tests:

• MRI of the pituitary gland to assess for tumors
• Urine test to measure cortisol levels
• Saliva test to measure cortisol levels
• Low-Dose Dexamethasone-Suppression Test (LDDST): This test requires the patient to take a small dose of cortisol late at night and have blood drawn the next morning to measure cortisol

If test results show that Cushing’s disease is present, the next step will be to uncover the source of high cortisol. This may require further testing, which often includes a petrosal sinus sampling. With petrosal sinus sampling, we test adrenocorticotropic hormone (ACTH) levels directly from the veins to determine where ACTH is being secreted from. 

How is Cushing’s disease treated?

There are a number of treatments available for Cushing’s disease. The best treatment option for each patient will depend on his or her specific case. 

Treatment may be as simple as making healthy lifestyle changes, or may require regular medication to lower cortisol levels, surgery to remove a tumor (if a tumor is the cause), or possible radiation therapy.

Healthy Lifestyle Changes to Treat or Prevent Cushing’s Disease

Cortisol is responsible for regulating the body’s metabolism and deciding how the body processes and uses nutrients for energy. When cortisol levels are too high, this has a direct and significant effect on the metabolism. (This is why excessive, rapid weight gain is the primary sign of Cushing’s disease.)

Changing your diet in the following ways can help reduce cortisol levels and reverse symptoms:

• Reduce your sodium intake: Sodium can raise blood pressure and worsen Cushing’s disease symptoms. Try to limit or eliminate fast food and processed food to better control sodium intake. 
• Lower your blood sugar: People with high cortisol levels often have high blood sugar too (Cushing’s Disease News). High blood sugar can lead to a number of health conditions that coincide with Cushing’s disease (such as bone loss, joint problems, and nerve damage), which can amplify symptoms and lead to even higher cortisol levels.
• Lower your cholesterol: High cholesterol is another symptom of Cushing’s disease. It’s best to try to avoid foods high in fat, and opt for foods high in fiber, like legumes, fruits, and vegetables to help lower cholesterol and ease symptoms associated with high cholesterol (hyperlipidemia).

It’s also important that we help patients manage any pre-existing conditions, such as chronic stress, anxiety, or depression. Your UCF Health physician will help you develop healthier techniques for dealing with stress, and work with you to minimize risk factors, alleviate symptoms, and manage or prevent Cushing’s disease.

At UCF Health, we believe in helping our patients live a healthy lifestyle by optimizing their diet, physical activity, and stress management techniques. Lifestyle changes are the most sustainable changes that tend to result in the best outcomes.  

If a case is too far progressed for lifestyle changes to be a sufficient solution on their own, medication or another procedure may be required. 

Medications to Treat Cushing’s Disease

There are various medications to control cortisol production, including ketoconazole, mitotane (Lysodren) and metyrapone (Metopirone). For people who have Cushing’s syndrome, as well as type 2 diabetes or glucose intolerance, mifepristone (Korlym, Mifeprex) may be prescribed. Mifepristone works by blocking the effects of cortisol in the body tissues.

Other possible medications include a twice-daily injection called pasireotide (Signifor), and an oral medication called osilodrostat (Isturisa). Either osilodrostat or pasireotide may be taken if surgery did not work or is unable to be performed.

Surgery to Treat Cushing’s Disease

For cases of Cushing’s disease that are caused by a tumor on the pituitary gland, surgery may be necessary to remove the tumor. This surgical procedure is called a transsphenoidal surgery and is performed by a neurosurgeon.

For patients who require surgery to treat Cushing’s disease,  our endocrinologists work with other specialists – including our own endocrine surgeon – to find the best solution. 

Radiation Therapy for Cushing’s Disease

Radiation therapy is another potential treatment route for Cushing’s disease. Radiation therapy is generally reserved for patients who didn’t benefit from surgery, because radiation treatment is not immediately effective. Depending on the type of tumor, radiation therapy could take years to remove the tumor (Cushing’s Support & Research Foundation).

If radiation therapy is the best treatment route for a patient, our doctors will collaborate with leading radiation oncologists to get them the care they deserve.

Can you prevent Cushing’s disease?

While you are not able to prevent tumors that cause Cushing’s disease, you can do your part to live a healthy life, eat a balanced diet, get exercise regularly, sleep at least 8 hours a night, and take care of your mental health to minimize other risk factors.

Practice healthy stress management and take up a relaxing new hobby, like yoga, meditating, painting, or exploring new trails with your dog. Any activity that lowers your cortisol and reduces stress can help prevent Cushing’s disease.

If you’re prescribed a glucocorticoid or steroid, have your primary care physician monitor your cortisol levels regularly. The earlier your doctor catches hypercortisolism, the easier it is to treat and manage. As Cushing’s disease progresses, the symptoms become more complex, and can be debilitating.

Early intervention of Cushing’s disease will help preserve muscle strength, prevent excessive weight gain, and prevent skin and hair changes.

Schedule an appointment with a leading endocrinologist today.

If you have Cushing’s disease or are at risk of the disease, we will develop a lifestyle and treatment plan to help you prevent the disease, minimize risk factors, and prevent, manage, or treat any symptoms.

We want to help you live your greatest, healthiest life, without uncomfortable symptoms. Our experienced endocrinologist, Dr. Amita Kathuria, is here to listen to your needs and develop a plan that helps you achieve optimal health.

Once you visit one of our healthcare providers, our new patient portal allows you to view lab results, send secure messages to your doctor, view statements and receipts, and manage prescriptions. 

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Sources:

https://www.aans.org/en/Patients/Neurosurgical-Conditions-and-Treatments/Cushings-Disease