Itsenko-Cushing syndrome (ICS)

Definition of the Disease and its Causes:

Itsenko-Cushing syndrome (ICS): A condition that develops due to elevated levels of cortisol in the blood, a hormone synthesized by the adrenal glands or introduced into the body through medications.

Cortisol is often considered a stress hormone, increasing in response to emotional stress, enabling the body to better cope with intense pressure situations. Its main functions include supporting stable blood pressure, controlling respiration, monitoring blood sugar levels, reducing inflammation, regulating electrolyte balance, controlling heart rate and muscle tension. Additionally, cortisol “shuts down” non-essential systems during stress periods, such as digestion and reproduction.

The regulation of cortisol levels in the blood involves cooperation between the hypothalamus, the pituitary gland (which produces ACTH), and the adrenal cortex. It begins with a signal from the hypothalamus to the pituitary gland to produce adrenocorticotropic hormone (ACTH). This hormone then travels through the blood to the adrenal cortex, stimulating cortisol production.

The causes of Itsenko-Cushing syndrome may be internal or external. Internal causes include:

1. Pituitary gland tumor: Typically benign, it secretes an excess amount of adrenocorticotropic hormone (ACTH), the most common cause of this syndrome (also known as Itsenko-Cushing disease).
2. Adrenal cortex tumor: Often benign, this is referred to as an adrenal cortex adenoma. Cancerous tumors in the adrenal cortex are rare.
3. ACTH-secreting tumor: Arises in organs that do not usually produce ACTH, such as the lungs, pancreas, thyroid gland, or thymus. These tumors can be benign or malignant.
4. Bilateral adrenal hyperplasia: Benign enlargement of adrenal tissue.

External causes of Itsenko-Cushing syndrome may include the prolonged use of glucocorticoids (hormones) in high doses to treat conditions such as rheumatoid arthritis, systemic lupus erythematosus, asthma, dermatitis, joint pain, and back pain. Some individuals may develop Itsenko-Cushing syndrome due to prolonged inappropriate external treatment using highly effective topical corticosteroids, especially if used over large areas of the skin (e.g., in the treatment of atopic dermatitis or psoriasis) or by using inhaled asthma medications containing glucocorticoids.

Symptoms of Itsenko-Cushing syndrome include:

1. Weight gain: Fat accumulates in the chest and abdomen, as well as in the back of the neck and shoulders, forming what is known as “buffalo hump,” while maintaining thinness in the limbs.
2. External signs: The face may appear flushed. The skin becomes thin and prone to bruising and abrasions.
3. Skin stretch marks (striae): Large, purplish stretch marks may appear on the abdomen, thighs, and arms.
4. Facial skin problems: These may include acne, blackheads, and oily skin.
5. Muscle weakness: Even difficulty climbing stairs and combing hair.
6. Psychological symptoms: These may include depression and mood swings.
7. Increased hair growth in women: Hirsutism.
8. Decreased libido and fertility problems: Sexual function and libido problems may appear.

Itsenko-Cushing syndrome is divided into several types:

According to the cause:

• Internal: Develops due to excess cortisol production in the body.
• External: Caused by treatment with glucocorticoids.

According to dependency on ACTH:

• ACTH-dependent: Occurs due to excess secretion of ACTH.
• ACTH-independent: Develops due to excess cortisol secretion without ACTH influence.

Complications of Itsenko-Cushing syndrome:
Without treatment, Itsenko-Cushing syndrome can lead to the development of complications.

The most important complications of Itsenko-Cushing syndrome include:

1. Osteoporosis: A condition characterized by decreased bone density and increased fragility. Unusual fractures, such as rib or foot fractures, may occur.
2. Hypertension: Increased blood pressure.
3. Steroid-induced diabetes: Glucose metabolism disorder due to increased adrenal cortex hormones.
4. Recurrent infectious diseases: Due to weakened immune system.
5. Loss of muscle mass: Muscle mass depletion.
6. Sexual dysfunction, infertility: Problems in sexual function and fertility.
7. Psychological disorders: Psychological disorders such as depression.

Diagnosing Itsenko-Cushing syndrome:

When there is suspicion of Itsenko-Cushing syndrome, it is advisable to consult a general practitioner. The doctor will conduct an interview to inquire about symptoms, perform a physical examination, measure weight and height, and inquire whether the patient is taking glucocorticoids.

For initial diagnosis, the doctor may order tests to measure free cortisol levels in urine throughout the day and total cortisol levels in saliva during the night.

Treatment of Itsenko-Cushing syndrome:

The treatment of Itsenko-Cushing syndrome depends on the underlying causes that led to its development.

1. If the condition is due to glucocorticoid intake, the dosage of the medication is gradually reduced for the patient. In some cases, hormonal therapy may even be discontinued entirely.
2. If Itsenko-Cushing syndrome is caused by a tumor, surgery is performed to remove it. In some cases, the doctor may suggest radiation therapy.
3. Medications may be prescribed to the patient to reduce the effect of cortisol on the body.
4. After successful surgical treatment, hypocorticism (cortisol deficiency) often occurs. Therefore, alternative therapy with non-steroidal anti-inflammatory drugs (cortisol) is regulated. This condition often resolves over time.
5. After treatment, the patient may need a long period of recovery and rehabilitation.

Sources:

• Raff H., Carroll T. Cushing’s syndrome: from physiological principles to diagnosis and clinical care // J Physiol. 2015. Vol. 593(3). P. 493–506. doi:10.1113/jphysiol.2014.282871