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Hypercortisolism - what it is, types and treatment of a dangerous disease. Endogenous hypercortisolism

Or Itsenko-Cushing syndrome is a disease that occurs as a result of long-term use of glucocorticoid hormones, or more precisely cortisol. Cortisol is an adrenal hormone produced by the pituitary gland (the front part of the brain). This hormone is indispensable in the functioning of the body. Its action is aimed at stabilizing blood pressure and insulin in the blood, reducing the inflammatory response of the immune system and regulating metabolic processes. But the most important function the hormone cortisol in regulating the body's response to emotional arousal, or more precisely to stress. For some people, in effect professional characteristics Cortisol levels are constantly elevated. These are usually athletes. But we should also not forget about women who are pregnant. Their levels increase significantly in the last trimester of pregnancy. Cortisol is also increased in those who lead an unhealthy lifestyle - drug addicts, smokers, alcoholics. People suffering mental disorders, are susceptible to hypercortisolism.

Causes of hypercortisolism

An increase in cortisol in the body is possible after:

  • Long-term treatment with glucocorticoid hormones, in particular prednisolone and dexamethasone.
  • Tumor formations and inflammatory processes occurring in the pituitary gland. This reason The formation of hypercortisolism is more common in the female sex than in the male sex.
  • Lung cancer and.
  • Benign tumors of the adrenal glands are adenomas.
  • Heredity.

Symptoms of hypercortisolism

The first symptom by which hypercortisolism or Itsenko-Cushing syndrome can be diagnosed is excessive body weight, in some cases obesity of the second and third degree. Moreover, the localization of fat deposits is atypical for ordinary obesity. Overweight concentrated in the abdomen, face and neck. Against this background, the limbs look excessively miniature. Moreover, this is not only an aesthetic problem, but also a real one. With hypercortisolism, the patient produces osteoporosis. This means that the bones begin to thin out, becoming brittle and brittle. Even minor trauma can cause a fracture.

Against the background of external signs, the patient feels constant fatigue And muscle weakness. The work of the central nervous system. The person experiences constant anger, anxiety and irritability.

In the first stages of the disease, hypercortisolism in humans increases blood pressure, blood sugar increases and, as a result, the first symptoms begin to appear. The patient constantly experiences extreme thirst and also needs frequent urination.

In females, hair begins to grow in all parts of the body - neck, chest, abdomen, thighs, face. Associated symptoms is a violation menstrual cycle or it stops altogether. Both men and women experience a decrease in sexual desire.

If you experience at least one of the above symptoms, you should immediately consult a doctor for full examination body.

Diagnosis of hypercortisolism

When a patient contacts medical clinic, the first thing he is diagnosed with is the degree of fat deposits. Next, the dermatologist analyzes the condition of the skin of the face and body. Afterwards, a diagnosis of the musculoskeletal system is necessary.

Then you need to carry out biochemical analysis blood to check for increased cortisol levels in the blood. In order to carry out this analysis, the patient needs to have a daily urine volume analyzed and a dexamethasone test done.

Also, a patient with a suspected diagnosis of hypercortisolism will be prescribed instrumental analyzes- This is a magnetic resonance imaging of the brain and adrenal glands.

Treatment of hypercortisolism

Three methods are used to treat hypercortisolism:

  • Medication;
  • Radiation therapy;
  • Surgical intervention.

The drug treatment method is aimed primarily at restoring hormonal levels in the body. To do this, the patient is prescribed medications that are aimed at reducing the synthesis of adrenal hormones. These may be drugs such as: mitotane, metyrapone, trilostane.

Drug treatment is carried out if it is impossible to carry out surgical intervention or as a means of restoring the body after surgery.

Radiation therapy is prescribed if the cause of hypercortisolism is benign tumor anterior section brain - pituitary gland. Radiation therapy affects brain cells, thereby reducing the synthesis of adrenocorticotropic hormone. In most cases, radiation therapy is combined with drug treatment and directly surgical intervention. But, nevertheless, it is better to use radiation therapy in combination with medications at the first stage of treatment - mitotane and metyrapone.

Surgical intervention for hypercortisolism involves the removal of a pituitary adenoma using latest technologies microsurgery. Improved well-being and general condition the body can be noted in the first week after surgical treatment. It is worth noting that in 80% of cases man walking is on the mend and is fully recovering after removal of the pituitary adenoma.

If a patient has both adrenal glands removed, he is advised to take glucocorticoids throughout his life as replacement therapy.

It's important to remember that timely treatment increases the chances of recovery. Do not ignore the body's signals screaming about the disease. After all, as medical statistics say, if you do not start treatment for hypercortisolism in the first 2 years from the moment of its occurrence, then in 50% of cases it is observed death.

Cushing's syndrome (hypercortisolism) is a long-term and chronic effect on the patient's body of an excessive amount of adrenal hormones, mainly cortisol. The syndrome can be caused by both internal diseases, and taking certain medications. According to statistics, women are susceptible this disease much more often than men (10 times more), and the main risk group is women aged 25 to 40 years. Due to a severe imbalance of hormones, the patient experiences pathological changes in appearance And biochemical processes body.

Cortisol, which is produced in excess in Cushing's syndrome, in this case can be safely called the hormone of death. IN large quantities Cortisol reduces the production of glucose, which is so necessary for most cells in our body, so due to a lack of glucose, some cell functions fade away or stop working altogether.

Causes of development of Cushing's syndrome.

There are many reasons that cause excessive production of adrenal hormones. There are three types of hypercortisolism: exogenous, endogenous and pseudo-Cushing's syndrome. Let's look at each of them separately and the reasons that most often cause them.

Exogenous hypercortisolism.

One of the most common causes of Cushing's syndrome is an overdose or long-term treatment steroids (glucocorticoids) for any other disease. Steroids are most often used to treat asthma, rheumatoid arthritis or immunosuppression during organ transplantation.

Endogenous hypercortisolism.

In this case, the cause of the disease is already internal violations in the functioning of the body. The most common cause, in approximately 70% of cases, of the development of the syndrome is Itsenko-Cushing's disease (not to be confused with Cushing's syndrome). In Itsenko-Cushing's disease, increased production adrenocorticotropic hormone from the pituitary gland (ACTH), which in turn stimulates the release of cortisol from the adrenal glands. This hormone can be produced by pituitary microadenoma or ectopic corticotropinoma. Ectopic malignant corticotropinoma can be located in the bronchi, testicles, and ovaries. Even more rarely, Cushing's syndrome occurs with a primary lesion of the adrenal cortex, for example, adrenal hyperplasia or malignant, benign tumors of the adrenal cortex.

Pseudo-Cushing's syndrome.

Sometimes the signs of the syndrome can be caused by completely different factors, but this is only a temporary phenomenon and does not mean that the person has true hypercortisolism. Common reasons development of pseudo-Cushing's syndrome - obesity, chronic alcohol intoxication, pregnancy, stress and depression, and sometimes even, which contain a mixture of estrogen and progesterone. An increase in cortisol levels in the blood can occur even in infants, when in their body along with breast milk alcohol comes in.

Signs and symptoms of Cushing's syndrome.

And obesity occurs in 90% of patients, with fat deposited on the face (moon-shaped, round face with blush on the cheeks), abdomen, chest and neck, and arms and legs look thin.

Muscle atrophy is very noticeable in shoulder girdle and legs. A rapid decrease in the patient is accompanied by. Together with obesity, this symptom creates great difficulties for patients when performing any physical activity. Sometimes there are painful sensations during squatting and lifting.

Very often, with hypercortisolism, the patient can observe thinning skin. The skin becomes marbled, dry and patchy. The patient often has wounds and cuts on his body, and in case of any violation of the integrity of the skin, they heal slowly.

Women with Cushing's syndrome very often develop. Hair starts to grow upper lip, chin and chest. All this is caused by the fact that the adrenal cortex begins increased production male hormones– androgens. In women, in addition to hirsutism, menstrual cycle disorders occur (), and in men, libido arises and decreases.

Osteoporosis is the most common symptom (90%) that occurs in patients with hypercortisolism. Osteoporosis first manifests itself in the form of pain in the bones and joints, and then spontaneous fractures of the ribs and limbs may occur. If the disease manifests itself in childhood, then the child’s growth development becomes noticeable.

With Cushing's syndrome, cardiomyopathy often develops mixed current. Arises this state against the background of arterial hypertension, electrolyte changes or catabolic effects of steroids on the myocardium. These disorders manifest themselves in the form of heart rhythm disturbances, high blood pressure, heart failure, which in most cases leads to the death of the patient.

Often, in approximately 10–20% of cases, patients with hypercortisolism experience steroid diabetes mellitus, which can be easily regulated with medications (glycemic drugs).

From the nervous system, signs such as lethargy, depression, euphoria, various disorders sleep and steroid psychosis.

Treatment for Cushing's syndrome (hypercortisolism).

Treatment of Cushing's syndrome is primarily aimed at eliminating the cause of hypercortisolism and balancing hormonal background in the body.

There are three treatment methods: medication, radiation therapy and surgery.

The main thing is to start treatment on time, because according to statistics, if treatment is not started in the first five years from the onset of the disease, then death occurs in 30–50% of cases.

Medicinal method of treatment.

At drug therapy The patient is prescribed drugs that reduce the production of hormones in the adrenal cortex. Often medications are prescribed to a patient complex therapy or if other treatment methods have not helped. To treat hypercortisolism, your doctor may prescribe mitotane, metyrapone, trilostane, or aminoglutethimide. Basically, these drugs are prescribed if surgery turned out to be ineffective or it is impossible to carry it out.

Radiation therapy.

This method is especially effective if the disease is caused by a pituitary adenoma. Radiation therapy, when exposed to the pituitary gland, causes it to reduce the production of adrenocorticotropic hormone. Radiation therapy is usually given along with medical or surgical treatment. Most often, it is in combination with medicines, applies this method, as it enhances the effect of drug treatment for Cushing's syndrome.

Surgical intervention.

For pituitary Cushing's syndrome, transsphenoidal revision of the pituitary gland and excision of the adenoma using microsurgical techniques are often used. Improvements come very quickly, and efficiency this method is 70 – 80%. If hypercortisolism is caused by a tumor of the adrenal cortex, then surgery is performed to remove this tumor. In isolated cases, especially in seriously ill people, both adrenal glands are removed, which is why the patient is prescribed lifelong use of glucocorticoids as replacement therapy.

Endogenous hypercortisolism (or Itsenko-Cushing's disease) is a neuroendocrine disease based on dysregulation of the hypothalamic-pituitary-adrenal system

Epidemiology.

  • - The incidence of endogenous hypercortisolism (Cushing's disease) is 2 new cases per year per 1 million population;
  • - More common in women aged 20-40 years

Etiological factors.

  • - Pituitary adenoma
  • - Infectious lesions of the central nervous system;
  • - Skull injuries;
  • - Primary “empty sella turcica”

Pathogenesis.

Infections, intoxications, tumors, mental and traumatic brain injuries lead to disruption of processes in the central nervous system, increasing the level of serotonin and reducing dopamine levels. This leads to increased secretion of corticoliberin by the hypothalamus and adrenocorticotropic hormone ACTH pituitary gland

The fact is that normally serotonin activates the corticotropin-corticotropin-releasing hormone system, and dopamine inhibits it. Therefore, an increase in the activating effect of serotonin and a decrease in the inhibitory effect of dopamine leads to excessive release of corticoliberin and ACTH. Enhanced Selection ACTH is a pathogenetic factor of endogenous hypercortisolism. It leads to increased secretion by the adrenal glands cortisol, aldosterone, androsterone.

At the same time, with endogenous hypercortisolism (Itsenko-Cushing's disease), the sensitivity of the hypothalamic-pituitary system to the action of corticosteroids is reduced, so the release of both ACTH and cortisol is simultaneously increased. At the same time, steroidogenesis enzymes are activated. All this leads to the development of clinical syndromes:

  • - asthenoadynamic,
  • - hypertensive,
  • - osteoporotic,
  • - dysplastic obesity,
  • - sexual dysfunction,
  • - impaired glucose tolerance,
  • - dermatological

Frequency of the main clinical signs of endogenous hypercortisolism (Itsenko-Cushing's disease)

Asthenoadynamic syndrome. Occurrence rate 96.5%. Symptoms: damage to the nervous and muscular systems, characterized by general weakness, decreased performance, unstable mood, and mental disorders.

Hypertensive syndrome. Occurrence rate 91.4%. Persistent arterial hypertension with its characteristic manifestations, complications and consequences.

Osteoporotic syndrome. Occurrence rate 88.4%. Bone pain, symptoms of secondary radiculitis against the background of systemic osteoporosis with predominant damage to the vertebrae, ribs, skull bones, pelvis; fractures.

Dysplastic obesity syndrome. Occurrence rate 93.6%. Uneven distribution of adipose tissue with excessive deposition in the abdomen, back, chest with relatively thin limbs.

Sexual dysfunction syndrome. Occurrence rate 72%. Menstrual irregularities, amenorrhea, infertility, hirsutism, lactorrhea in women. Impotence, sterility in men.

Impaired glucose tolerance syndrome. Occurrence rate 62%. In some cases, diabetes mellitus occurs (34.9%)

Dermatological syndrome. Occurrence rate 54%. The skin is thin, dry, cyanotic, with acne on the chest and back. In the abdomen, shoulders, hips, and chest there are wide purplish-cyanotic stretch marks, hyperpigmentation in areas of friction.

Itsenko-Cushing syndrome and disease (hypercortisolism) is a symptom complex that develops when the adrenal glands produce excessive corticosteroid hormones. If the disease occurs as a result of disorders in the hypothalamic-pituitary system, which lead to an increase in the production of ACTH (adrenocorticotropic hormone) and, as a consequence, cortisol, then they actually speak of Itsenko-Cushing’s disease. If the appearance of symptoms of the disease is not associated with the functioning of the hypothalamic-pituitary system, then we're talking about about Itsenko-Cushing syndrome.

Causes of hypercortisolism

In some cases, the cause of hypercortisolism is a pituitary adenoma.
  1. Exogenous or drug-induced hypercortisolism can be caused long-term use drugs containing glucocorticoid hormones (prednisolone, dexamethasone, etc.) in the treatment of any diseases, for example bronchial asthma, systemic lupus erythematosus, etc. In such cases, it is necessary to reduce the dose of drugs to a maintenance dose (minimum acceptable) to control this disease.
  2. Ectopic ACTH production is a condition in which tumors of various origins outside the pituitary gland they produce adrenocorticotropic hormone, for example, thymus, or.
  3. Tumors of the adrenal glands, and most often excess production of cortisol occurs with benign tumors of these glands (adenoma).
  4. Familial form of Itsenko–Cushing syndrome. In most cases, this disease is not inherited, but there is hereditary syndrome multiple endocrine neoplasia, in which a person has an increased likelihood of developing tumors, including those of the adrenal glands.
  5. – a benign tumor of the pituitary gland, leading to hyperproduction of ACTH and, as a consequence, excessive production of cortisol by the adrenal cortex. Hypercorticism, resulting from damage to the pituitary gland, is actually called Itsenko-Cushing's disease. According to statistics, this disease is five times more common in women, and tumors of other endocrine glands are often detected simultaneously.

Symptoms of hypercortisolism

Itsenko–Cushing syndrome has quite pronounced external signs, by which the doctor may suspect this disease.

The overwhelming majority of patients exhibit obesity, characteristic of hypercortisolism. The distribution of fat occurs according to the dysplastic (Cushingoid) type: an excess amount of fatty tissue is determined on the back, abdomen, chest, and face (moon face).

Muscle atrophy, volume reduction muscle mass, decreased muscle tone and strength, especially noticeable violation of the tone of the gluteal muscles. “Sloping buttocks” is one of the typical signs of the disease. Atrophy of the anterior muscles abdominal wall may eventually lead to hernias of the white line of the abdomen.

Skin changes. In patients, the skin becomes thinner, has a marbled tint, is prone to dryness, flakes, and there are areas increased sweating. A vascular pattern is visible under the thinned skin. Striae appear - purple-purple stretch marks, most often appearing on the skin of the abdomen, thighs, mammary glands, and shoulders. Their width can reach several centimeters. In addition, many different rashes such as acne and spider veins. In some cases, with Itsenko-Cushing's disease, hyperpigmentation is observed - dark and light spots that stand out in color appear on the skin.

One of the severe complications of Itsenko–Cushing syndrome is associated with thinning bone tissue, resulting in fractures and bone deformations, and over time, patients develop scoliosis.

Heart dysfunction is expressed in the form of cardiomyopathy. Heart rhythm disturbances and arterial hypertension occur, and over time, patients develop heart failure.

In 10–20% of patients, steroid diabetes mellitus is detected, although there may be no damage to the pancreas and significant changes blood insulin concentrations.

Excessive production of adrenal androgens leads to hirsutism in women - hair growth male type. In addition, sick women experience menstrual irregularities, including amenorrhea.

Diagnosis and treatment of Itsenko–Cushing syndrome


In urine with this pathology it is found increased level hormone cortisol.

The examination begins with screening tests: a urine test to determine the level of daily excretion free cortisol, tests with dexamethasone (a glucocorticosteroid drug), which help identify hypercortisolism, as well as conduct preliminary differential diagnosis syndrome and Itsenko-Cushing's disease. Further studies are needed to identify pituitary adenomas, adrenal tumors or other organs and complications of the disease ( ultrasound diagnostics, radiography, computer and magnetic resonance imaging, etc.).

Treatment of the disease depends on the results of the examination, i.e. on the specific area in which it is detected pathological process, leading to the occurrence of hypercortisolism.

Today the only truly effective method Treatment of Itsenko-Cushing's disease resulting from pituitary adenoma is surgical (selective transsphenoidal adenomectomy). This neurosurgery indicated when identifying a pituitary adenoma with a clear localization established during examination. Usually this operation gives positive results in the vast majority of patients, restoration of the normal functioning of the hypothalamic-pituitary system occurs quite quickly, and in 70–80% of cases there is stable remission diseases. However, relapse may occur in 10–20% of patients.

Surgical treatment of Itsenko–Cushing's disease is usually combined with radiation therapy, as well as by the method of destruction of hyperplastic adrenal glands (since the mass of the glands increases as a result of prolonged hormonal imbalance).

If there are contraindications to surgical treatment pituitary adenomas ( serious condition patient, presence of severe concomitant diseases etc.) is carried out drug treatment. Drugs are used that suppress the secretion of adrenocorticotropic hormone (ACTH), an increase in the level of which leads to hypercortisolism. Against the background of such treatment, if necessary, it is necessary to carry out symptomatic therapy antihypertensive, hypoglycemic drugs, antidepressants, treatment and prevention of osteoporosis, etc.

If the cause of hypercortisolism is tumors of the adrenal glands or other organs, then their removal is necessary. When removing the affected adrenal gland in order to prevent hypocorticism in the future, patients will need long-term replacement hormone therapy under constant control doctor

Adrenalectomy (removal of one or two adrenal glands) is rarely used in recent years, only when it is impossible to treat hypercortisolism by other means. This manipulation leads to chronic adrenal insufficiency, requiring lifelong hormone replacement therapy.

All patients who have been or are undergoing treatment for this disease require constant monitoring by an endocrinologist, and, if necessary, other specialists.

Which doctor should I contact?

If these symptoms appear, you should contact an endocrinologist. If, after examination, a pituitary adenoma is detected, the patient is treated by a neurologist or neurosurgeon. If complications of hypercortisolism develop, treatment is indicated by a traumatologist (for fractures), a cardiologist (for increased blood pressure and rhythm disturbances), and a gynecologist (for disorders of the reproductive system).