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Cushing syndrome

Hypercortisolism; Cortisol excess

 

Cushing syndrome is a disorder that occurs when your body has a high level of the hormone cortisol.

Causes

 

The most common cause of Cushing syndrome is taking too much glucocorticosteroid medicine. This form of Cushing syndrome is called exogenous Cushing syndrome . Prednisone, dexamethasone, and prednisolone are examples of this type of medicine. Glucocorticoids mimic the action of the body's natural hormone cortisol. These drugs are used to treat many conditions such as asthma, skin inflammation, cancer, bowel disease, joint pain, and rheumatoid arthritis.

Other people develop Cushing syndrome because their body produces too much cortisol. This hormone is made in the adrenal glands. Causes of too much cortisol are:

  • Cushing disease , which occurs when the pituitary gland makes too much of the hormone ACTH. ACTH then signals the adrenal glands to produce too much cortisol. A pituitary gland tumor can cause this condition.
  • Tumor of the adrenal gland
  • Tumor elsewhere in the body that produces corticotropin-releasing hormone (CRH)
  • Tumors elsewhere in the body that produce ACTH ( ectopic Cushing syndrome )

 

Symptoms

 

Symptoms vary. Not everyone with Cushing syndrome has the same symptoms. Some people have many symptoms while others have hardly any symptoms.

Most people with Cushing syndrome have:

  • Round, red, full face ( moon face )
  • Slow growth rate (in children)
  • Weight gain with fat accumulation on the trunk, but fat loss from the arms, legs, and buttocks (central obesity)

Skin changes that are often include:

  • Skin infections
  • Purple marks (1/2 inch or 1 centimeter or more wide) called striae on the skin of the abdomen, thighs, and breasts
  • Thin skin with easy bruising

Muscle and bone changes include:

  • Backache, which occurs with routine activities
  • Bone pain or tenderness
  • Collection of fat between the shoulders and above collar bone
  • Rib and spine fractures caused by thinning of the bones
  • Weak muscles, especially of the hips and shoulders

Women with Cushing syndrome often have:

  • Excess hair growth on the face, neck, chest, abdomen, and thighs
  • Periods that become irregular or stop

Men may have:

  • Decreased or no desire for sex
  • Erection problems

Other symptoms that may occur with this disease:

  • Mental changes, such as depression, anxiety, or changes in behavior
  • Fatigue
  • Headache
  • Increased thirst and urination

 

Exams and Tests

 

The health care provider will perform a physical exam and ask about your symptoms and the medicines you are taking. Tell the provider about all medicines you have been taking for the past several months. Also tell the provider about shots that you received at a provider's office.

Laboratory tests that may be done to diagnose Cushing syndrome and identify the cause are:

  • Blood cortisol levels
  • Blood sugar
  • Saliva cortisol levels
  • Dexamethasone suppression test
  • 24-hour urine for cortisol and creatinine
  • ACTH level
  • ACTH stimulation test  (rarely)

Tests to determine the cause or complications may include:

  • Abdominal CT
  • ACTH
  • Pituitary MRI
  • Bone mineral density

 

Treatment

 

Treatment depends on the cause.

Cushing syndrome caused by corticosteroid use:

  • Your doctor will instruct you to slowly decrease the medicine dosage. Stopping the medicine suddenly can be dangerous.
  • If you cannot stop taking the medicine because of disease, your high blood sugar, high cholesterol levels, and bone thinning or osteoporosis should be closely monitored.

With Cushing syndrome caused by a pituitary or a tumor that releases ACTH (Cushing disease), you may need:

  • Surgery to remove the tumor.
  • Radiation after removal of a pituitary tumor in some cases.
  • Cortisol replacement therapy after surgery and possibly for the rest of your life.

With Cushing syndrome due to an adrenal tumor or other tumors:

  • You may need surgery to remove the tumor.
  • If the tumor cannot be removed, you may need medicines to help block the release of cortisol.

 

Outlook (Prognosis)

 

Removing the tumor may lead to full recovery, but there is a chance that the condition will return.

Survival for people with ectopic tumors depends on the tumor type. Untreated, Cushing syndrome can be life threatening.

 

Possible Complications

 

Health problems that may result from Cushing syndrome include any of the following:

  • Diabetes
  • Enlargement of pituitary tumor
  • Fractures due to osteoporosis
  • High blood pressure
  • Kidney stones
  • Serious infections

 

When to Contact a Medical Professional

 

Call your provider if you have symptoms of Cushing syndrome.

 

Prevention

 

If you take a corticosteroid, know the signs and symptoms of Cushing syndrome. Getting treated early can help prevent any long-term effects of Cushing syndrome. If you use inhaled steroids, you can decrease your exposure to the steroids by using a spacer and by rinsing your mouth after breathing in the steroids.

 

 

References

Ferri FF. Cushing's disease and syndrome. In: Ferri FF, ed. Ferri's Clinical Advisor 2016 . Philadelphia, PA: Elsevier Mosby; 2016:385-386.

Nieman LK, Biller BM, Findling JW, et al. Treatment of Cushing's syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab . 2015;100:2807-2831. PMID: 26222757 www.ncbi.nlm.nih.gov/pubmed/26222757 .

Stewart PM, Newell-Price JDC. The adrenal cortex. In: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology . 13th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 15.

 
  • Endocrine glands - illustration

    Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the pace of chemical activity in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).

    Endocrine glands

    illustration

    • Endocrine glands - illustration

      Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the pace of chemical activity in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).

      Endocrine glands

      illustration

    A Closer Look

     

      Talking to your MD

       

        Self Care

         

          Tests for Cushing syndrome

           

             

            Review Date: 10/28/2015

            Reviewed By: Brent Wisse, MD, Associate Professor of Medicine, Division of Metabolism, Endocrinology & Nutrition, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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