Cushing Syndrome

Cushing syndrome (also called hypercortisolism) is a condition that occurs when a person’s body is exposed to too much of a hormone called cortisol or a medication similar to cortisol for too long. The syndrome is usually caused by taking certain medicines that mimic cortisol. Other causes include tumors that make the body produce excess cortisol. Cushing syndrome includes a range of treatable symptoms. In most cases, the syndrome is curable. NICHD is one of the many federal agencies that support and conduct research on the causes of Cushing syndrome, ways to diagnose it earlier, and better treatments.

About Cushing Syndrome

Cushing syndrome occurs when the body’s tissues are exposed to too much of the hormone cortisol or a medication similar to cortisol for too long.1 The syndrome is named after Harvey Cushing, the brain surgeon who first described the condition.2

Cortisol is an essential hormone for life. The body’s two adrenal glands produce the hormone cortisol daily for our routine activities and a higher amount of cortisol in response to stress or when the cortisol levels in the blood are lower than they should be.

In the right amount, cortisol helps the body with several vital tasks:

  • Maintaining blood pressure and heart function
  • Controlling the immune system
  • Turning food into energy
  • Raising blood sugar levels as needed
  • Controlling bone formation

If the body makes too much cortisol or gets too much from external sources, like medications, Cushing syndrome may occur. Whether you develop Cushing syndrome depends on factors such as how much medication you take and for how long or how large a tumor grows before it is detected and treated.

Citations

  1. Stewart, P. M., & Newell-Price, J. D. C. (2016). The adrenal cortex. In S. Melmed, K. S. Polonsky, P. R. Larsen, & H. M. Kronenberg (Eds.), Williams Textbook of Endocrinology, 13th ed. (pp 490–555). Philadelphia, PA: Elsevier.
  2. Doyle, N. M., Doyle, J. F., & Walter, E. J. (2017). The life and work of Harvey Cushing 1869–1939: A pioneer of neurosurgery. Journal of the Intensive Care Society, 18(2), 157–158. Retrieved February 7, 2019, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5606407/

What causes Cushing syndrome?

Cushing syndrome can develop for two reasons:

  • Medication that contains glucocorticoid, which is similar to cortisol1,2
  • tumor in the body that makes the adrenal gland produce too much cortisol1,2

Citations

  1. Nieman L. K., & Ilias, I. (2005). Evaluation and treatment of Cushing syndrome. Journal of American Medicine, 118(12), 1340–1346. PMID 16378774.
  2. Nieman, L. K., Biller, B. M. K., Findling, J. W., Newell-Price, J., Savage, M. O., Stewart, P. M., & Montori, V. M. (2008). The diagnosis of Cushing syndrome: An Endocrine Society clinical practice guideline. Journal of Clinical Endocrinology and Metabolism, 93(5), 1526–1540. Retrieved March 2, 2017, from https://academic.oup.com/jcem/article/92/1/10/2597828 
  3. Batista, D. L., Courcoutsakis, N., Riar, J., Keil, M. F., & Stratakis, C. A. (2008). Severe obesity confounds the interpretation of low-dose dexamethasone test combined with the administration of ovine corticotrophin-releasing hormone in childhood Cushing syndrome. Journal of Clinical Endocrinology and Metabolism, 93(11), 4323-4330. PMID 18728165.

What are the symptoms of Cushing syndrome?

Cushing syndrome can cause a range of symptoms.1,2 The symptoms might also be similar to those of other conditions.3

Physically, someone with Cushing syndrome might:

  • Be heavy or obese above the waist but have thin arms and legs
  • Have a round, red face, sometimes referred to as a moon face
  • Have a fat lump between the shoulders, sometimes called a buffalo hump
  • Have weak muscles or bones including osteoporosis, bone pain, and fractures
  • Have skin changes, including:
    • New or worsened acne or skin infections
    • Reddish-purple stretch marks called striae (pronounced STRY-ee). These are usually about half an inch wide and can appear on the abdomen, buttocks, thighs, arms, and breasts.
    • Thin, fragile skin that bruises easily and heals poorly

Additional symptoms can include the following:

  • Children may get heavier but grow more slowly than their peers.
  • Women may have more hair on their face, neck, chest, abdomen, and thighs. They may experience menstrual problems, such as irregular or stopped periods.
  • Men may have lower sex drive, experience impotence, and become less fertile.

The following less common symptoms also may develop:

  • Mental changes such as depression, anxiety, moodiness, or behaving differently
  • Severe fatigue
  • Headaches
  • Thirst and increased need to urinate
  • High blood pressure
  • High blood sugar
  • High cholesterol and triglycerides

Overall, the symptoms that most strongly hint at Cushing syndrome are the fatty deposit on the upper back/neck, fat around the abdomen, weakness in muscles closest to the torso (such as in the shoulders and hips), wide striae (skin stripes), bruising without being bumped, unexplained osteoporosis, and—in children—slower growth in height and more weight gain that may be mistaken for obesity.2,3

Citations

  1. Batista, D. L., Riar, J., Keil, M., & Stratakis, C. A. (2007). Diagnostic tests for children who are referred for the investigation of Cushing syndrome. Pediatrics120(3), e575-e586. Retrieved March 14, 2017, from http://pediatrics.aappublications.org/cgi/pmidlookup?view=long&pmid=17698579 
  2. Nieman, L. K., & Ilias, I. (2005). Evaluation and treatment of Cushing’s syndrome. Journal of American Medicine, 118(12), 1340–1346. PMID 16378774.
  3. Nieman, L. K., Biller, B. M. K., Findling, J. W., Newell-Price, J., Savage, M. O., Stewart, P. M., & Montori, V. M. (2008). The diagnosis of Cushing’s syndrome: An Endocrine Society clinical practice guideline. Retrieved March 2, 2017, from https://academic.oup.com/jcem/article/93/5/1526/2598096 

How do healthcare providers diagnose Cushing syndrome?

Diagnosing Cushing syndrome can be complex and difficult. This syndrome is easier to recognize once it develops fully, but healthcare providers try to diagnose and treat it before it gets to that point. No single test can definitively diagnose the condition, so healthcare providers often use several steps to diagnose Cushing syndrome. If you are being checked for Cushing syndrome, the tests may occur in the following order1,2:

Citations

  1. Nieman, L. K., Biller, B. M. K., Findling, J. W., Newell-Price, J., Savage, M. O., Stewart, P. M., & Montori, V. M. (2008). The diagnosis of Cushing’s syndrome: An Endocrine Society clinical practice guideline. Retrieved March 2, 2017, from https://academic.oup.com/jcem/article/93/5/1526/2598096 
  2. Boscaro, M., & Arnaldi, G. (2009). Approach to the patient with possible Cushing’s syndrome. Journal of Clinical Endocrinology and Metabolism, 94(9), 3121–3131. Retrieved April 9, 2019, from https://www.ncbi.nlm.nih.gov/pubmed/19734443
  3. MedlinePlus. (n.d.). Dexamethasone suppression test. Retrieved March 2, 2017, from https://medlineplus.gov/ency/article/003694.htm

Is there a cure for Cushing syndrome?

Untreated Cushing syndrome can be life-threatening. Fortunately, most people with the syndrome are treated and cured.1

However, after successful treatment of the syndrome, some health problems may continue. For instance, your bones may continue to be weak. People who no longer have Cushing syndrome may be more likely to have high blood pressure, diabetes, and mental health issues, such as depression and anxiety.1

People whose Cushing syndrome was caused by a tumor need to have regular checkups for the rest of their lives to check for additional tumors. For many adults with Cushing disease (the term used for Cushing syndrome caused by a pituitary adenoma), surgical removal of the tumor is successful. In rare cases, some of the tumor cells are left and the adenoma can grow back.1

Citations

  1. Nieman, L. K., Biller, B. M. K., Findling, J. W., Murad, M. H., Newell-Price, J., Savage, M. O., & Tabarin, A. (2015). Treatment of Cushing’s syndrome: An Endocrine Society clinical practice guideline. Journal of Clinical Endocrinology & Metabolism, 100(8), 2807–2831. Retrieved March 3, 2017, from https://academic.oup.com/jcem/article/100/8/2807/2836065 

What are the treatments for Cushing syndrome?

Treatment for Cushing syndrome depends on the cause of the extra cortisol in the body.1,2,3

Citations

  1. Graversen, D., Vestergaard, P., Stochholm, K., Gravholt, C. H., & Jørgensen, J. O. (2012). Mortality in Cushing’s syndrome: A systematic review and meta-analysis. European Journal of Internal Medicine, 23(3), 278–282. Retrieved April 9, 2019, from https://www.ncbi.nlm.nih.gov/pubmed/22385888
  2. MedlinePlus. (2019). Cushing’s syndrome. Retrieved April 9, 2019, from http://www.nlm.nih.gov/medlineplus/cushingssyndrome.html
  3. Nieman, L. K., Biller, B. M. K., Findling, J. W., Murad, M. H., Newell-Price, J., Savage, M. O., & Tabarin, A. (2015). Treatment of Cushing’s syndrome: An Endocrine Society clinical practice guideline. Journal of Clinical Endocrinology & Metabolism, 100(8), 2807–2831. Retrieved March 3, 2017, from https://academic.oup.com/jcem/article/100/8/2807/2836065 
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