What is Cushing's syndrome?

Cushing's Syndrome is an excess of cortisol in the body that results in a variety of changes, which are discussed in more detail below.

How does it occur?

Cortisol is a hormone normally produced by the adrenal glands to help regulate various processes in the body. There are multitude of causes that can result in higher than normal levels of cortisol in the body. The production of cortisol is directly controlled by another hormone called ACTH, which is made in the pituitary. The levels of ACTH in turn are controlled higher up in the brain by the hypothalamus. By understanding this pathway of hypothalamus > pituitary (ACTH) > cortisol (made by the adrenal gland), one can see how an increase at any of these levels can result in elevated cortisol.

Tumors in the brain that overproduce ACTH, thereby causing excess cortisol production, is a common cause of Cushing's syndrome. There are other tumors in the body, such as lung tumors, that can also overproduce ACTH.

Tumors in the adrenal gland can lead to overproduction of cortisol directly. The most common cause of Cushing's syndrome is from long-term steroid medication usage. Steroids are used to treat many diseases and chronic therapy with them can lead to Cushing's syndrome.

What are the symptoms?

The symptoms are wide ranging as cortisol has many effects on the body. High levels of cortisol can cause increased weight most notably in the face ("moon facies"), chest and the back ("buffalo hump").

Other findings include diabetes, muscle weakness, fatigueability, osteoporosis leading to bone fractures, easy bruisability, high blood pressure, irritability and emotional instability, purplish lines on the skin (esp. the abdomen), menstrual changes and virilizing signs.

How is it diagnosed?

After asking questions and performing a physical exam, the doctor will order the necessary blood tests if he suspects Cushing's syndrome. The diagnosis is made through demonstration of elevated levels of cortisol.

Elevated levels of cortisol can be detected by either an early morning blood test (before eating) or by a 24-hour collection of urine. After high levels of cortisol have been documented, further blood tests can be done to help determine the cause.

Other useful and common tests may include a chest x-ray, CT of the brain and CT of the abdomen to look for tumors.

How is it treated?

Treatment of Cushing's syndrome is usually best accomplished by surgery. For those with a tumor in the pituitary, adrenal or other organ site, surgical removal is the best mode of treatment. Most patients will also require supplemental steroid medication for a few months. Radiation of the pituitary tumor is also possible, but has a much less successful cure rate.

For those patients unable to undergo an operation, medical therapy with ketoconazole, metyrapone or mitotane can be instituted. These drugs work by slowing down the production of cortisol.

Prognosis for the disease varies depending on the cause. Those patients with adrenal or pituitary tumors who have complete removal of the tumor by surgery have cure rates above 90 percent.

How can I take care of myself?

For those patients diagnosed with Cushing's syndrome, it is very important to be followed by a physician closely and follow instructions from the doctor. This becomes especially important for those patients who undergo surgery. After the operation, compliance with medications and close follow-up will be critical for the health of the patient.




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