Brief overview
- Symptoms: Muscle breakdown and increased fat storage, wound healing disorders, parchment skin, increased susceptibility to infections, male pattern hair in women (hirsutism), reproductive disorders, growth disorders in children, psychological problems (such as depression), increased cholesterol, high blood pressure, osteoporosis.
- Causes: Excessive intake of cortisone-containing drugs (exogenous Cushing’s syndrome) or overproduction of cortisol in the adrenal glands (endogenous Cushing’s syndrome); endogenous Cushing’s syndrome is usually due to a benign or malignant tumor (for example, of the pituitary or adrenal glands).
- When to see a doctor? When one or more of the above symptoms occur
- Treatment: discontinuation of cortisone-containing drugs (exogenous Cushing’s syndrome), tumor treatment by surgery, radiotherapy, chemotherapy, or adrenostatics (endogenous Cushing’s syndrome).
What is Cushing’s syndrome?
Doctors speak of a “syndrome” when several signs of illness (symptoms) are present at the same time. In the case of Cushing’s syndrome, there are even many symptoms.
The high cortisol level is triggered either by cortisone-containing drugs (exogenous Cushing’s syndrome) or – in very rare cases – by hormone-producing tumors (endogenous Cushing’s syndrome). If a tumor in parts of the brain (hypothalamus or pituitary gland) is responsible, we speak of central Cushing’s syndrome or Cushing’s disease.
How Cortisol Works
Cortisol is a vital hormone that has numerous functions. It is also known as the “stress hormone” because it is produced in greater quantities when the body is exposed to permanently increased demands.
As a “stress hormone”, cortisol activates degradative (catabolic) metabolic processes to provide the body with energy to cope with extraordinary stress.
- It indirectly (via gene expression) provides sugar (through gluconeogenesis in the liver), proteins (mainly through muscle breakdown) and fat. In the middle of the body (abdomen, neck, face) it simultaneously causes increased fat storage.
- Furthermore, cortisol causes a breakdown of collagen, connective tissue and bone substance, which in excess leads to a breakdown (atrophy) of the skin and a decrease in bone density.
- Finally, cortisol still plays an important immunosuppressive role by inhibiting the immune response and inflammatory reactions to a certain extent. This is also the reason why cortisol is of great importance as a drug.
How does Cushing’s syndrome manifest itself?
In accordance with the diverse functions of cortisol in the body, the symptoms of Cushing’s syndrome also manifest themselves in a wide variety of places:
- Full moon face / moon face: Typical for Cushing’s syndrome is also a round and red face due to fat storage.
- Bull neck (buffalo neck): Fat is also increasingly deposited in the neck area.
- Altered blood values: The amount of cholesterol in the blood is often increased in Cushing’s syndrome. Frequent changes in the blood count, rarely potassium deficiency.
- Increased blood pressure: Many people with Cushing’s syndrome have increased blood pressure.
- Muscle weakness and reduced bone density: In the context of Cushing’s syndrome, there is a breakdown of muscle and bone substance. Thus, muscle mass and bone density decrease. Under certain circumstances, osteoporosis develops.
- Increased susceptibility to infections: due to the general suppression of the immune system in Cushing’s syndrome, affected individuals are susceptible to infections – for example, more frequent colds
- Psychological changes: In some cases, Cushing’s syndrome is also associated with psychological changes, such as depression.
- Reduced potency: Men with Cushing’s syndrome often develop impotence.
- Decreased growth: In children, Cushing’s syndrome impairs growth, via indirect inhibition of growth hormone.
What is the life expectancy with Cushing’s syndrome?
If a tumor causes Cushing’s syndrome (endogenous Cushing’s syndrome) and it is treated successfully, the prognosis for a restored quality of life is good.
Despite successful treatment with a decrease in symptoms, endogenous Cushing’s syndrome may result in increased mortality due to associated conditions such as cardiovascular disease or infection.
However, if left untreated, the disease is fatal in many cases within a few months or years due to its numerous concomitant diseases.
Causes
When considering the cause, the first step is to distinguish exogenous Cushing’s syndrome from endogenous Cushing’s syndrome. Exogenous means that it is brought about from the outside. In contrast, endogenous Cushing’s syndrome results from a malfunction or disease in the body.
Exogenous Cushing’s syndrome
The active ingredient cortisone (after being converted into cortisol in the body) has an anti-inflammatory and generally immunosuppressive effect.
It is therefore used, for example, to treat chronic inflammatory bowel diseases (Crohn’s disease, ulcerative colitis), asthma, multiple sclerosis and many autoimmune diseases. In such cases, cortisone is usually administered systemically, for example as a tablet or infusion.
The dose of glucocorticoids that triggers Cushing’s syndrome in a patient is called the Cushing’s threshold.
Endogenous Cushing’s syndrome
Endogenous Cushing’s syndrome is also based on an excess of glucocorticoids or cortisol. In this case, however, it originates in the body itself. Endogenous Cushing’s syndrome is much rarer than the exogenous variant, with only about two to three patients per million population. Women are affected much more frequently than men.
Cortisol is produced in the adrenal glands, more precisely in the so-called adrenal cortex. Other hormones are also produced there, such as androgens (male sex hormones like testosterone) and aldosterone (important for regulating water and sodium balance).
It stimulates the activity of the adrenal cortex. Put simply, this means that the more ACTH is produced in the pituitary gland, the more hormones (mainly cortisol) the adrenal cortex produces.
The self-control of cortisol formation now occurs with the help of a regulatory circuit between CRH, ACTH and cortisol:
If there is sufficient cortisol circulating in the blood, it throttles CRH and ACTH release in the hypothalamus and pituitary gland, respectively – and thus indirectly further cortisol production.
The higher the cortisol level in the blood, the more CRH and ACTH are inhibited and the less new cortisol is produced in the adrenal cortex.
Depending on where the disorder is localized, endogenous Cushing’s syndrome is divided into ACTH-dependent and ACTH-independent variants:
ACTH-dependent Cushing’s syndrome.
ACTH-dependent Cushing’s syndrome is responsible for about 85 percent of all cases of endogenous Cushing’s syndrome. It is due to an excess of the pituitary hormone ACTH. This stimulates the adrenal cortex to produce excessive cortisol.
Regardless of whether the increased ACTH formation ultimately originates from the pituitary gland or from the hypothalamus – in both cases one speaks of central Cushing’s syndrome (also called Cushing’s disease), since the cause of the ACTH excess lies in the central nervous system (brain).
These ACTH-producing tumors include, for example, small cell bronchial carcinoma (a form of lung cancer) and rare intestinal tumors. Occasionally, ectopic Cushing’s syndrome is also due to a CRH-producing tumor: it overstimulates ACTH production by the pituitary gland and, in turn, indirectly stimulates cortisol production.
ACTH-independent Cushing’s syndrome
The cause is usually a cortisol-producing tumor of the adrenal cortex. In adults, this is usually a benign tumor (adenoma), while in children it is more often a malignant tumor (carcinoma).
In young children, such a tumor of the adrenal gland is also the most common cause of endogenous Cushing’s syndrome. Most are under 5 years of age at the time of diagnosis. In children over 7 years of age, central ACTH-dependent Cushing’s syndrome is more common.
A distinction must be made between these forms of Cushing’s disease and an excess of cortisol triggered by alcohol consumption – alcohol-induced “pseudo-Cushing’s syndrome”. It is assumed that here an increased release of CRH by the hypothalamus is the cause. This form of the disease usually disappears after a longer period of abstinence from alcohol.
When should you see a doctor?
The earlier Cushing’s syndrome, or its causes, are treated, the greater the chances that it will recede and that no permanent damage will remain.
What does the doctor do?
This is followed by a physical examination. Among other things, the doctor measures the patient’s blood pressure and weight. He also inspects the patient’s skin. With the help of various tests, it can be determined whether there is actually an excess of cortisol (hypercortisolism) in the blood and, if so, where it comes from:
- 24-hour collection urine: The patient’s urine excreted over the course of 24 hours is collected in order to then determine the concentration of free cortisol in it. In the case of Cushing’s syndrome, the measurement result is significantly increased.
There are other tests that are applicable in the workup of Cushing’s syndrome. These include the insulin hypoglycemia test (to detect elevated cortisol levels), as well as the dexamethasone long test and the CRH stimulation test: The latter two are helpful in distinguishing between the different forms of Cushing’s syndrome (central, paraneoplastic, or adrenal Cushing’s syndrome).
Treatment
In the case of exogenous Cushing’s syndrome, all cortisone-containing drugs are discontinued if possible. However, this should not be done abruptly!
If cortisone preparations are taken for longer than a few days, this inhibits the body’s own cortisol production of the adrenal glands. This occurs via the pituitary and pituitary gland control organs, which release less CRH and ACTH due to the increased cortisol level in the blood.
It makes sense to discuss the exact procedure with your doctor. He will give you a precise scheme at what intervals you may reduce the dose and how far, so that your body is always sufficiently supplied with cortisol.
A few weeks after surgery, a blood test is recommended to check whether hormone levels have normalized and thereby assess whether further surgery is necessary.
If surgery is not an option, a tumor may also be treated with radiation therapy or chemotherapy. Sometimes so-called adrenostatics are prescribed – drugs that inhibit cortisol production in the adrenal glands.
What can be done yourself?
In addition to the actual therapy of hypercortisolism, its consequences and accompanying symptoms such as obesity, cardiovascular diseases and diabetes can be positively influenced by a healthy, balanced diet and sufficient exercise.
If possible, avoid alcohol, caffeine and nicotine, as these stimulants may promote secondary and concomitant diseases.
Regular exercise reduces weight, facilitates the regulation of blood sugar, lowers blood pressure, strengthens the bones and thus also makes an important contribution to Cushing’s management.
Ask your doctor for recommendations on an optimized diet for Cushing’s and what form of exercise is appropriate in your individual case.
Frequently asked questions about Cushing’s syndrome
What is Cushing’s syndrome?
How does Cushing’s syndrome manifest itself?
Cushing’s syndrome is manifested by weight gain, especially on the face and abdomen, thin skin that bleeds easily, blue or purple stretch marks, and muscle weakness. Other signs include high blood pressure, high blood sugar levels, increased body hair, fatigue and mood swings. Women often experience menstrual irregularities, and men experience erectile dysfunction.
What triggers Cushing’s syndrome?
What can be done about Cushing’s syndrome?
To treat Cushing’s syndrome, the overproduction of the hormone cortisol must be stopped. If a tumor in the adrenal glands or pituitary gland is the cause, surgery or radiation therapy is often necessary. Certain medications can also reduce or block cortisol production.
Is Cushing’s syndrome dangerous?
How long does Cushing’s syndrome last?
Cushing’s syndrome is a chronic disease. The course of the disease depends on the cause and the effectiveness of treatment. It may take several weeks for the symptoms to disappear completely. Sometimes it even takes several months or years. In some cases, there is also persistent fatigue and weakness.
How does Cushing’s syndrome make you feel?
What happens if Cushing’s syndrome is not treated?
Without treatment, Cushing’s syndrome causes serious health problems such as diabetes, hypertension, osteoporosis, muscle weakness, and increased risk of infection. In addition, mental illnesses such as depression and anxiety, erectile dsyfunction in men, and menstrual cycle disorders in women occur. In rare cases, a life-threatening situation can occur if Cushing’s syndrome is not treated.
