Cushing’s syndrome

Cushing’s syndrome

Definition:

A disease caused by increased production of cortisol or by excessive use of cortisol or other steroid hormones.

Alternative Names:

Hypercortisolism

Causes, incidence, and risk factors:

Cushing's syndrome is a condition that results from an excess of cortisol, a hormone produced by the adrenal glands. The most common cause of Cushing's syndrome is Cushing's disease caused by excessive production of adrenocorticotropic hormone (ACTH) by the pituitary gland. ACTH stimulates the adrenal glands to produce cortisol.

Cushing's syndrome can be caused by a tumor of the pituitary gland, a tumor of the adrenal gland, a tumor somewhere other than the pituitary or adrenal glands (ectopic Cushing's syndrome), or by long-term use of corticosteroids (drugs commonly used to treat conditions, such as rheumatoid arthritis and asthma).

Risk factors are adrenal tumor or pituitary tumor, chronic therapy with corticosteroids, and being female.

See also:

Symptoms:

moon face (round, red, and full)
buffalo hump (a collection of fat between the shoulders)
central obesity with protruding abdomen and thin extremities
weight gain
weakness
backache
headache
acne or superficial skin infections
thin skin with easy bruising
thirst
increased urination
purple striations on the skin of the abdomen, thighs, and breasts
mental changes
impotence or cessation of menses
facial hair growth

Additional symptoms that may be associated with this disease:

Signs and tests:

Tests to confirm high cortisol level:

cortisol, urine
dexamethasone suppression test
serial serum cortisol levels do not show diurnal variations

Tests to determine the cause:

ACTH
cranial MRI or cranial CT scan may show pituitary tumor
abdominal CT may show adrenal mass

General findings:

glucose test is elevated
potassium test may be low
white blood cell count may be elevated

Treatment:

Treatment depends upon the cause of the disorder. In Cushing's syndrome caused by drug therapy with corticosteroids, the drug must be slowly decreased under medical supervision.

In Cushing's disease caused by a pituitary tumor, surgery to remove the tumor is recommended. Sometimes, radiation is needed as well. Hydrocortisone (cortisol) replacement therapy is needed after surgery, and sometimes forever.

Cushing's syndrome caused by an adrenal tumor is usually treated by surgical removal of the tumor. If the tumor cannot be removed, certain medications can suppress the secretion of cortisol.

In Cushing's syndrome caused by a tumor secreting ACTH, resection of the tumor is the best way to treat the Cushing's syndrome.

Cortisol replacement therapy is indicated after surgery until cortisol production resumes. In some cases, life-long therapy with cortisone drugs becomes necessary.

Expectations (prognosis):

Tumor removal may lead to full recovery, but there is a chance of recurrence. Survival for people with ectopic tumors depends upon the overall outcome associated with the particular tumor type. Untreated, Cushing's syndrome can lead to death.

Complications:

diabetes mellitus
high blood pressure
serious infections
fractures due to osteoporosis
kidney stones
enlargement of pituitary tumor

Calling your health care provider:

Call your health care provider if symptoms develop that indicate Cushing's syndrome.

Prevention:

Cushing's syndrome may be prevented by an awareness of the associated symptoms so that early detection can be made.

Review Date: 2/14/2002
Reviewed By: Stephanie Fish, M.D., Division of Endocrinology, University of Pennsylvania Medicial Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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