Cushing's Syndrome (Cushing's Disease, Hypercortisolism)

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Definition

Cushing's syndrome is a hormonal disorder caused by prolonged exposure to the hormone cortisol. It is called Cushing's disease when it is caused by a pituitary tumor. All other causes of the condition are termed Cushing's syndrome.

Causes

Prolonged or excess exposure to cortisol as a result of:

  • Long-term use of corticosteroid hormones such as cortisone or prednisone
  • Tumor or abnormality of the adrenal gland, which causes the body to produce excess cortisol
  • Tumor or abnormality of the pituitary gland, which causes the body to produce excess cortisol
  • Rarely, tumors of the lungs, thyroid, kidney, pancreas, or thymus gland produce hormones that trigger the syndrome

Pituitary and Adrenal Glands

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Risk Factors

A risk factor is something that increases your chance of developing a disease or condition.

Risk factors include:

  • Chronic use of corticosteroid medicines
  • Age: 20-50 years
  • Sex: female (much more common in women)

Symptoms

Although symptoms may vary, common symptoms of Cushing's syndrome are:

  • Weight gain of the upper body and trunk
  • Face shaped like a moon
  • Skin changes:
    • Darkening of the skin
    • Purple stretch marks
    • Easy bruising
  • Excess hair growth or acne in women
  • Menstrual disorders, especially infrequent or absent periods
  • Diminished fertility and libido
  • High blood pressure
  • Water retention or swelling
  • High blood sugar or diabetes
  • Tiredness or fatigue
  • Personality changes or mood swings
  • Muscle weakness
  • Osteoporosis or brittle bones
  • Skeletal growth retardation in children
  • Increased thirst
  • Frequent urination
  • Psychosis
  • Low back pain

Diagnosis

The doctor will ask about your symptoms and medical history, and perform a physical exam.

Tests may include: 24-hour Urinary Free Cortisol LevelUrine is collected for 24 hours, and then tested for cortisol. Late-evening Cortisol Saliva/Blood LevelA saliva or blood sample is collected at around 11 pm and tested for cortisol level. Dexamethasone Suppression TestA synthetic cortisol, called dexamethasone, is taken by mouth either overnight or for several days. Depending on the length of the test, or the dose of dexamethasone administered, blood or urine cortisol levels are measured at specific intervals. Tests to Determine Cause of Cushing's SyndromeCRH stimulation testACTH levelHigh-dose dexamethasone suppression test X-rays and Scans These tests may show whether there is a tumor in the pituitary or adrenal glands or another area of the body. Common imaging tools include: MRI scan a test that uses magnetic waves to make pictures of the inside of the body CT scan a type of x-ray that uses a computer to make pictures of structures inside the body Chest x-ray which may detect tumors in the lungs TreatmentTreatment of Cushing's syndrome depends on the cause. Treatments include: Surgical removal of tumorRadiation for some persistent tumorsGradual withdrawal of cortisone-type drugs (under close medical supervision)Drugs to suppress adrenal gland function PreventionWork with your doctor to keep use of corticosteroid drugs to a minimum.
RESOURCES: American Academy of Family Physicianshttp://familydoctor.org Cushing's Support and Research Foundationhttp://www.CSRF.net CANADIAN RESOURCES: Canadian Family Physicianhttp://www.cfpc.ca/cfp/ Health Canadahttp://www.hc-sc.gc.ca/index_e.html References: Arnaldi G, Angeli A, Atkinson AB, Bertagna X, et al. Diagnosis and complications of Cushings syndrome: a consensus statement. J Clin Endocrinolo Metabo . 2003;88:5593-5602. Cushing's Syndrome and Cushing's Disease. Am Fam Physician . 2000;62(5):1133. Cushing's Syndrome. National Institute of Diabetes and Digestive and Kidney Diseases website. Available at: http://www.niddk.nih.gov/health/endo/pubs/cushings/cushings.htm . Accessed October 7, 2005. Diez JJ and Iglesias P: Pharmacological therapy of Cusings syndrome: drugs and indications. Mini Rev Med Chem 2007;7:467-80. Fauci AS, Braunwald E, Isselbacher KJ, et al. Harrison's Principles of Internal Medicine . 14th ed. New York, NY: The McGraw-Hill Companies; 2000. Makras P, Toloumis G, Papadoglas D et al: the diagnosis and differential diagnosis of endogenous Cusings syndrome. Hormones 2006;5:231-50.
Yaneva M, Mosnier-Pudar H, Dugue MA, Grabar S, Fulla Y, Bertagna X. Midnight salivary cortisol for the initial diagnosis of Cusings syndrome of various causes. J Clin Endocrinol Metab . 2004;89:3345-3351. Last reviewed January 2008 by David Juan, MDPlease be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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