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Cushing's syndrome

Cushing's syndrome

 


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Condition
Cushing's syndrome
Class
Endocrine disorders
Description
Cushing's syndrome, also known as hypercortisolism, is a hormonal or endocrine disorder caused by prolonged exposure of the body's tissues to an excess of corticosteroid hormones.
Causes
Most people develop the symptoms of Cushing's syndrome because they take high doses of corticosteroids such as dexamethasone, hydrocortisone or prednisolone for the treatment of asthma, rheumatoid arthritis or other inflammatory diseases. Others develop the symptoms of Cushing's syndrome because they produce too much of their own corticosteroid hormone called cortisol (hydrocortisone). The body's cortisol is normally produced by glands that sit on top of the kidney (the adrenal glands) in response to the action of another hormone called adrenocorticotropic hormone or ACTH that is released by the pituitary gland in the brain. A tumour of the pituitary gland may result in the release of large amounts of ACTH which in turn causes the release of large amounts of cortisol from the adrenal glands.

To distinguish between the two, the terms Cushing's disease and Cushing's syndrome are used. Cushing's disease is used to describe symptoms caused by an excess of cortisol due to a pituitary tumour. Cushing's syndrome is used to describe symptoms caused by the unwanted side effects of drug treatment with corticosteroids. Cushing's syndrome is also used to describe symptoms caused by an excess of cortisol produced because of a tumour of the adrenal gland itself, or produced because of the release of ACTH from other parts of the body (ectopic ACTH release) such as oat cell or small cell cancers in the lung and carcinoid tumours.
Symptoms
The most common symptom shown by Cushing's syndrome patients is excessive weight gain resulting in a rounding of the face (sometimes called moon face) and an obese torso with fat deposited particularly above the collarbone and on the back of the neck (sometimes called buffalo hump). The arms and legs tend to remain slender. A reddened face with thin skin and visible blood vessels is also characteristic of Cushing's syndrome. Wounds tend to heal poorly and bruises appear easily. Bluish-red stretch marks resulting from weakened connective tissue appear over the abdomen, thighs, buttocks, arms, armpits and breasts.

Women with Cushing's syndrome commonly develop excessive hair growth (hirsutism) on the face, back, chest, abdomen and thighs. Menstrual disorders are also common with irregular or absent periods. Men with Cushing's syndrome often experience decreased fertility along with a diminished or absent sex drive.

High blood pressure, resulting from a build up of fat in the arteries (atherosclerosis) occurs in 85% of patients. Bones often become brittle and easily break or crack due to a decrease in bone mass (osteoporosis). High blood sugar levels, psychiatric disturbances (mood swings, depression, panic attacks and forgetfulness), severe weakness, fatigue, thirst and swollen feet are also characteristic of this disorder.

If Cushing's syndrome is left untreated it can be fatal; death occurring as a result of cardiovascular complications or infections.
Treatment
Treatment of Cushing's syndrome relates directly to the cause of the excess of the corticosteroid. If the cause is the long-term use of high doses of steroids to control other inflammatory conditions such as asthma or rheumatoid arthritis, the doctor may try to reduce the dose of the corticosteroid. The dose will be reduced gradually over a few weeks to a level that controls the inflammatory condition without producing symptoms of Cushing's syndrome. If lowering the dose of corticosteroid does not work, the doctor may decide to prescribe an alternative product to control your asthma or rheumatoid arthritis and gradually withdraw the corticosteroid completely.

If there is an excess cortisol production, either because of a pituitary tumour, an adrenal tumour or because of a tumour elsewhere in the body, surgery, chemotherapy or radiotherapy may be used to destroy the tumour. If there is no success with these methods, drugs that inhibit the synthesis of steroids by the adrenal glands, for example, ketoconazole, mitotane, metyrapone, trilostane and aminoglutethimide, may be used to stop overproduction of corticosteroids. However, as the body cannot function without corticosteroids, replacement therapy with corticosteroid tablets or injections has to be given to keep corticosteroids at normal levels to avoid adrenal insufficiency. Also, as the pituitary gland is responsible for the secretion of a number of other hormones, for example growth hormone or thyroid stimulating hormone, replacement therapy with these and other hormones may also be required to correct any imbalance caused by treatment.
When to consult your pharmacist
Let your pharmacist know if you have been diagnosed with corticosteroid-induced Cushing's syndrome and your doctor is trying to reduce your dose of corticosteroids. As you gradually withdraw from the corticosteroids, the pharmacist will need to know that perhaps the strength of your tablets has been reduced or that you are taking a lower dose. If when withdrawing corticosteroids, you develop an infection, experience a traumatic event or come into contact with someone who has got chickenpox, shingles or measles, tell your pharmacist immediately. You will be advised to see your doctor who may recommend a temporary reintroduction of corticosteroid treatment until you recover.
When to consult your doctor
It is often difficult to diagnose Cushing's syndrome because the symptoms may develop gradually and can resemble the symptoms of many other medical conditions. If your doctor suspects that you have Cushing's syndrome that has not been caused by drug treatment with corticosteroids, you will be referred to a specialist in hormone disorders (an endocrinologist). The endocrinologist will perform a variety of tests and examinations to measure your levels of cortisol and ACTH and, if their levels are found to be too high, will perform further tests and scans to locate the source of the problem. If your doctor suspects that your symptoms are caused by the long term use of high doses of corticosteroids to treat other conditions, your corticosteroid dose will probably be reduced or gradually withdrawn.
Living with Cushing's syndrome
When your doctor or endocrinologist tells you that you have Cushing's disease or Cushing's syndrome you may greet the news with mixed reactions. You may feel disappointment to learn that you have an endocrine problem, while at the same time feel relief to know that there is a reason for the way that you feel and the changes that have happened to your body. Talk things over with your doctor or endocrinologist so that you understand your condition and the treatments that will be given. Between appointments, keep a diary of the way you feel and the things that you do not understand so you remember to ask the questions that matter most to you on your next visit. The healthcare professionals are there to help you and the more you understand your condition, the better your chances of making a near complete recovery.

If you have Cushing's syndrome as the result of an excess of corticosteroids used to treat inflammatory diseases, it is important that you follow your doctor's advice about gradually reducing the corticosteroids. Do not be tempted to stop the corticosteroids suddenly. Not only may this cause the sudden and life threatening return of your condition, for example an asthma attack, but it also carries the risk of causing what is known as an 'adrenal crisis'. Cortisol is essential for life and health. While taking or receiving prescribed corticosteroids, the body reduces its natural production of cortisol. If the prescribed corticosteroids are suddenly withdrawn, the body cannot adapt quickly enough to produce the levels that the body needs. As a result, blood pressure may fall suddenly, leading to shock, coma and death.

Similarly if you have had surgery to treat Cushing's disease, replacement therapy must be taken to restore the corticosteroids and any other hormones while the body adapts to compensate. None of these hormones must be withdrawn without the advice of an endocrinologist.

In times of stress, for example following an accident, an infection or because of surgery, the body requires additional amounts of corticosteroids to cope. Consequently while taking corticosteroids or when gradually withdrawing corticosteroids, the dose may need to be increased or the withdrawal of the corticosteroid may need to be reversed temporarily until the stress has passed. If you become ill or require surgery, including dental surgery, tell your doctor, dentist or anyone treating you so that they are aware of your treatment and can decide if it is necessary to adjust your dose of corticosteroids.

Whether you have Cushing's disease or Cushing's syndrome it is important to stay healthy. As far as diet is concerned, eat three balanced meals a day at normal breakfast, lunch and dinner times. Reduce meal portions, cut down on fatty foods and salt and eat more pasta, brown rice, wholemeal bread, vegetables and fruit. Boil or grill food rather than frying it. Besides helping to control weight, a healthy diet will help reduce the risk of heart disease, high blood pressure and diabetes.

Exercise regularly. Although you may feel too tired at first, build up your level of fitness gradually and you will find that in a short space of time not only will you be able to exercise more vigorously, but you will also feel better, lighter in mood, less depressed, and more able to relax and sleep at night.

If you smoke, make every effort to give. Ask your doctor or pharmacist for help and advice. Stopping smoking is the single most significant factor in reducing the risk of heart disease.

Maintain your independence, but do not struggle alone. Let family and friends help.
Further information
ACTH - The Association for Cushings Treatment and Help is a patient support group run by patients. It aims to provide advice and support throughout the treatment process and during the following years.

Association for Cushings Treatment and Help
54 Powney Road
Maidenhead
Berkshire
S16 6EQ
Tel: 01628 670389
Email: cushingsacth@btinternet.com
www.acth-cushings.org.uk

Reviewed on 11 October 2010


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