Cushing's disease & syndrome
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Table of contents
Overview
Cushing's syndrome is caused by inappropriate and chronic exposure to excess cortisol levels. The source of this excess cortisol can be endogenous or exogenous (i.e., medication).
When the excess cortisol is caused by a pituitary adenoma (a tumor of the pituitary gland in the brain), the patient's condition is defined as Cushing's disease. Cushing's disease represents 70% of cases of endogenous Cushing's syndrome.
Typical clinical features of Cushing's syndrome are central obesity with abdominal skin stretch marks (but with thin arms and legs and proximal muscle weakness), fat pad buffalo hump, moon face, emotional instability and depression, severe fatigue and weakness, dyslipidemia, diabetes, hypertension and osteopenia.
Because other disorders have similar signs and symptoms, Cushing's syndrome can be difficult to diagnose (especially endogenous Cushing's syndrome), with a diagnosis time of more than 6 years.
Cushing's syndrome occurs in less than 1 in 10,000 people in the European Union, representing fewer than 51,000 patients. Cushing's syndrome is more common in women than in men and represents 75% of the population.
The generally accepted treatment goal for patients is to normalize cortisol levels. Typical treatments include surgery and/or medical interventions. With proper treatment, the progression of the disease can be stopped and symptoms reduced.
Diagnosis
The first step in diagnosing Cushing's syndrome is to check the medications a patient is taking. Then the second step consists of various tests, including:
- The measurement of cortisol levels in urine that will show if the body is producing too much cortisol. Urine can be collected over a 24-hour period.
- Saliva test late at night: cortisol levels vary throughout the day. In people without Cushing's syndrome, this level drops significantly in the evening. If this level is too high late at night, doctors may suspect Cushing's syndrome.
- The Dexamethasone Suppression Test (DST) is also commonly used to diagnose Cushing's syndrome and consists of measuring blood cortisol levels after taking Dexamethasone.
Symptoms
Patients with Cushing's syndrome may develop the following signs and symptoms, which can vary from patient to patient:
- Rapid weight gain
- Central obesity
- Emotional instability and depression
- Severe fatigue and weakness
- Osteopenia (low bone density)
- Dyslipidemia (too high or too low blood lipid levels)
- Moon face (medical sign in which the face takes on a rounded appearance)
- Hirutism (i.e. excessive hair growth on the face or body)
- Striae
- Buffalo Hump
- Irregular menstruation in women
- Acne
Causes
Cushing's syndrome can have an endogenous or exogenous origin.
Endogenous Cushing's syndrome is caused by excessive cortisol production. Under normal circumstances, ACTH (adrenocorticotropic hormone) is secreted by the pituitary gland to stimulate the release of cortisol by the adrenal glands (located above the kidneys).
There are 3 different causes of endogenous Cushing's syndrome:
- In pituitary Cushing's disease, also known as Cushing's disease, a pituitary adenoma induces excessive production of ACTH, leading to increased production of cortisol, and this represents 70% of endogenous Cushing's syndromes
- In adrenal Cushing's, the increased production of cortisol is caused by an adrenal tumor.
- In diseases such as small cell lung cancer, ACTH can be produced outside the pituitary-adrenal cortex and affect the production of cortisol.
Exogenous Cushing's syndrome (or iatrogenic) is caused by the use of glucocorticoids (e.g., Prednisone) to treat other diseases (such as asthma).
Video
Patients Association
AdrenalNet - https://bijniervereniging-nvacp.nl
Cushing patient association: adrenal association NVCAP - https://bijniervereniging-nvacp.nl/
