Treatment of cushing’s disease abroad
Cushing disease is a rare endocrine disease as a result of pituitary adenoma or hyperplasia. The incidence in different countries is from 1 to 3 cases per million of population per year. Usually pathology develops between the age of 20-40. Cushing's disease is 5 times more common for women than for men.
Causes of Cushing’s disease
The causes and mechanism of Cushing's disease development have not yet been established. There are only a few risk factors that increase the likelihood of this pathology.
Among them are:
- Head injury
- Arachnoiditis, encephalitis or other inflammatory lesions of the CNS
- For women - childbirth
The majority of symptoms develop due to increased cortisol secretion. It stimulates the production of the pituitary adrenocorticotropic hormone (ACTH). Regulation based on the principle of feedback which means that the more cortisol in the blood, the less ACTH is produced. Reducing the level of cortisol, ACTH secretion by the pituitary gland increases and leads to increased production of cortisol.
Cushing's disease is characterized by the formation of pituitary tumor. As a result, production of ACTH is enhanced, and a feedback mechanism stops working. Cortisol levels remain elevated permanently.
Other hormone levels change that are characteristic for Cushing's disease:
- Reduction of the secretion of growth hormone ("HGH"), gonadotropin, thyroid-stimulating hormone
- Increase of prolactin, aldosterone, androgens in the blood vessels
As a result of hormonal imbalance rate of protein breakdown predominates over the rate of its synthesis. The blood level of potassium reduces and bones are derived of calcium.
Cushing’s disease - Symptoms
Like most hormonal imbalances, Cushing's disease causes symptoms on the part of all organs and systems.
Sexual disorders arethe early symptoms of the Cushing's disease. Women experience the menstrual cycle disorders, there are secondary sexual characteristics, and infertility develops. In the case of pregnancy significantly increases the risk of miscarriage and premature birth. Men can experience the erectile dysfunction and hypoactive sexual desire disorder (decreased libido).
Skin changes. It becomes thin and dry. There are stretch marks on the chest and extremities venous pattern becomes clearly visible. A blue tinge on the skin of hands and feet is possible. In places where there is friction hyperpigmentation occurs (excessive staining). Hair falls out, including women (androgenetic alopecia). Ulcers appear on the face.
Obesity isobserved in 92% of patients. Characteristic for Cushing’s disease is the deposition of fat in the face, neck and torso. The limbs remain thin.
The cardiovascular system. Almost all patients have elevated blood pressure and quickly develop cardiac arrhythmias, shortness of breath, swelling. Phenomena of heart failure are progressing. This is the reason the majority of deaths happen for patients with Cushing's disease.
Osteoporosis develops among 80% of patients due to "washout" of calcium from the bones and is included in the number of late manifestations of Cushing's disease. The consequence of osteoporosis is the increased fragility of bones. Chronic pain that a person can experience after exercise or at rest.
Infectious complications. When levels of glucocorticoids are elevated the immunity is reduced. Therefore, patients often have erysipelas, pyelonephritis, boils, and other nonspecific inflammatory processes.
Violation of carbohydrate metabolism. Glucose tolerance is rare for half of patients with the Cushing's disease. 15% of patients can have diabetes.
Cushing’s disease - Diagnostics
Diagnostics of Cushing’s disease is based on the characteristic clinical manifestations of the illness, and also laboratory and instrumental methods of diagnostics.
Determination of hormones level in the blood and urine. The concentration of ACTH and cortisol is elevated, as well as the circadian rhythm of their secretion is disrupted.
Adrenal glands research. Half of the patients have increased adrenal glands, but even if there is no adrenal hypertrophy, they are usually marked with seal.
Imaging techniques that are used to evaluate the status of these organs:
- Ultrasonic tomography
- Radioisotope imaging
X-ray methods. X-ray of the skull reveals the pituitary adenoma. The main X-ray symptom is an increased sella (skull structure, where the pituitary gland is located). But it is only observed among 10% of cases with Cushing's disease. The remaining 90% are microadenomas. They can be detected in 60% of cases by computed tomography.
Treatment of Cushing’s disease abroad
Treatment can be pathogenic or symptomatic. Pathogenic therapy aims to normalize the level of hormones in the blood. Symptomatic is aimed at eliminating specific clinical manifestations of disease (obesity, osteoporosis, heart disease, disorders of carbohydrate metabolism).
Transsphenoidal prostatectomy. Pituitary tumor is removed with the help of neurosurgery. This is the primary method of treatment to achieve a stable improvement for the majority of patients. The operation is performed using microsurgical instruments.
Radiation therapy. The irradiation of the intermediate-pituitary region is performed. This method is good because it’s not invasive, but its effectiveness is low. Radiation therapy is applied only for mild and moderate forms of the Cushing’s disease.
Adrenalectomy is a palliative surgery, when both adrenal glands are removed for the severe stage of the disease. One adrenal gland is removed for mild stages. Afterwards, lifelong replacement hormonal therapy will be needed. Adrenalectomy can be combined with radiation therapy if necessary.
Medication treatment is used for inhibition of the cortisol formation or as a symptomatic therapy. Method is combined with other treatments and also indicated for patients who are contraindicated the surgery.
Cushing’s disease - Prognosis
With the natural course of the disease without any treatment, the five-year survival is only 50%. When unilateral or bilateral adrenalectomy is performed, it increases to 85%.
In the case of high-quality surgical treatment with removal of pituitary adenoma complete remission of the disease occurs in 90% of cases. At the same time, level of secretion of ACTH and cortisol normalizes and normal metabolism restores.
For moderate and severe cases, even after a successful radical treatment the residual symptoms are observed:
- Arterial hypertension
- Heart failure
- Renal insufficiency
The most favourable prognosis can be made for a mild stage of Cushing’s disease with a short course for patients up to 30 years old. They have the ability to work and enjoy quality of life. The life expectancy of such patients is not reduced.