What is Cushing's Syndrome?
Harvey Cushing was the first to describe in 1932, Cushing's Disease, a disease presenting with hypersecretion of cortisol, that will later be grouped with other pathologies with different aetiologies but with same presentation, under what today we call Cushing's syndrome¹.
Cushing's Syndrome is a condition that occurs when the body produces an excess of cortisol over a long period of time².
Cortisol is produced by the adrenal glands, two small glands on top of the kidneys. The pituitary gland in the brain sends signals to the adrenal glands to produce cortisol³.
Cortisol is sometimes called "the stress hormone" because it helps the body responds to stress. It also helps to maintain blood pressure, regulate blood glucose (blood sugar), reduce inflammation, and turn the food into energy. Nevertheless, an excess of cortisol can cause harmful effects3³
Endogenous Cushing1s syndrome can be caused by the overproduction of cortisol due to tumours in the pituitary (What was first described by Harvey Cushing¹) and adrenal glands². However exogenous Cushing's syndrome can result from high dose steroid intakes for a long period of time².
What are the symptoms of Cushing's syndrome?
CS is clinically unmistakable when it is fully developed, but the spectrum of clinical presentation is wide with insidious onset therefore the diagnosis can be challenging in mild cases4.
The most common clinical signs of CS are:2,4
· weight gain
· thin arms and legs
· a round face
· increased fat around the base of the neck
· a fatty hump between the shoulders
· easy bruising
· wide purple stretch marks, mainly on the abdomen, breasts, hips, and under the arms
· weak muscles
Children with Cushing's syndrome tend to have obesity and grow more slowly than other children2,4. Women may have excess hair on their face, neck, chest, abdomen, and thighs. Menstrual periods may become irregular or stop5. Men may have decreased fertility with lowered interest in sex and may have erectile dysfunction6,7.
How common is cushing's syndrome?
Cushing's syndrome caused by tumours in the pituitary or adrenal glands is rare8.
Each year, there are only 0.7–2.4 new cases per million. It usually occurs in people over the age of 40 years, and it affects four times as many women than men7.
Cushing's syndrome can be difficult to diagnose because the symptoms are also seen in more common diseases.
CS can be initially investigated using one of the following four tests based on individual patient characteristics4:
· 24-hour urinary free cortisol (UFC) test (at least two measurements);
· Late-night salivary cortisol (LNSC) test (two measurements);
· Overnight 1-mg dexamethasone suppression test (DST); or
· Longer low-dose DST (2-mg/day for 48 hours).
Magnetic resonance imaging (MRI) and computed tomography (CT) scans may also be used to confirm the diagnosis8.
CS treatment aims to rapidly normalise the cortisol levels, and consequently, to improve the metabolic and biochemical abnormalities associated with the disorder9.
This is commonly done by removing the tumour that is causing cortisol levels to rise. If this does not work, other options include radiotherapy, medical therapy and removing the adrenal glands7. If you are a patient, please contact your physician for more information.
If you are a healthcare professional from the EU, you can find further information on the treatment guidelines for Cushing's syndrome here.
Want more information about Cushing's syndrome?
Hear the experiences of three patients with Cushing's syndrome: Watch Sammy, Ida or Lily's story here.
The Pituitary Network Association (www.cushings.org) have created a site full of useful information on Cushing's syndrome.
Additional information on Cushing's syndrome could be found on www.cushingshub.com. This website is dedicated for healthcare professionals.
References
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