Cushing's disease is a disease of the endocrine system that develops as a result of damage to the hypothalamic-pituitary system and increased production of ACTH hormone by the adrenal cortex. This pathology is characterized by multisymptom. Most often, the disease develops in women. Patients suffering from Itsenko-Cushing's disease are diagnosed with obesity, arterial hypertension, diabetes mellitus and many other specific manifestations.
To confirm the assumption of this disease, the patient will have to undergo a series of biochemical blood tests, hormone tests, X-ray of the spinal column and chest, CT scan of the pituitary and adrenal glands. Treatment of pathology is carried out with medication, radiation and surgery.
What is this disease?
In medicine, two concepts are distinguished: disease and Itsenko-Cushing's syndrome. In the second case, we are talking about the primary manifestations of hypercorticism. Both the disease and Itsenko-Cushing's syndrome have the same symptom complex, but at the same time they have a different nature of origin.
The development of the syndrome is based on the increased production of the human hormone - glucocorticosteroid, which is caused by hyperplastic tumors of the adrenal cortex. Basically, such a malfunction in the endocrine system occurs due to an increase in the size of corticosteromas, glucosteromas, adenocarcinomas. Hypercorticism, provoked by prolonged use of synthetic steroids, can also become the cause of Itsungo-Cushing's syndrome. In rare cases, the prerequisites for the development of this pathology are cancers of the ovary, lungs, pancreas, intestines, thyroid gland. Against the background of these malignant processes, ectopic ACTH syndrome develops with similar clinical manifestations.
Unlike the syndrome, Cushing's disease originates at the level of the hypothalamic-pituitary system, and the adrenal glands and other glands of the endocrine system are involved in the pathological process in the later stages. The disease occurs in the fairer sex several times more often than in men, while most often the disease affects women of childbearing age. The course of the disease is characterized by serious neuroendocrine disorders, including metabolic disorders, neurological symptoms, malfunctions of the cardiovascular, digestive and excretory systems.
Provocative factors
The causes of Cushing's disease lie in the presence of pituitary adenoma, producing an excessive amount of adrenocorticotropic hormone. In the case of a tumor lesion of this gland of the brain, adenocarcinoma, micro- or macroadenomas are found in patients. Often the occurrence of these neoplasms is associated with the consequences of infections and damage to the central nervous system (encephalitis, arachnoiditis, meningitis), traumatic brain injury, intoxication. In medical practice, there have been cases when Cushing's disease in women developed against the background of hormonal disorders caused by pregnancy, childbirth, menopause.
Pathogenesis
The mechanism of development of Itsenko-Cushing pathology is a violation of the hypothalamic-pituitary-adrenal interconnection, as a result of which too much ACT hormone is produced. In turn, the increased synthesis of this substance serves as an explanation of a whole string of adrenal and extrarenal reactions in the body:
- In the adrenal glands, the synthesis of glucocorticoids and male hormones increases, while the secretion of mineralocorticoids decreases.
- Excessive amounts of glucocorticoids negatively affect protein-carbohydrate metabolism, which leads to atrophy of connective tissue, hyperglycemia and insulin resistance, which precedes the development of hormonal diabetes.
- Improper fat metabolism is a favorable condition for the development of obesity.
- Hypokalemia and high blood pressure are a consequence of reduced mineral activity.
- The catabolic effect of hormones on bone tissue prevents the full absorption of calcium in the digestive tract, so osteoporosis develops.
- Increased production of androgens (male hormones) causes ovarian dysfunction and disruption of the female reproductive system.
Severity
There are several forms of the clinical course of the disease Itsenko-Cushing. The disease has three degrees of severity:
- Easy. The disease is accompanied by mild symptoms: the menstrual cycle can be saved, and signs of osteoporosis can be completely absent.
- Medium. The symptoms of Cushing's disease are almost impossible to confuse with the manifestations of other diseases, however, at this stage the disease does not progress, the general condition of the patient remains satisfactory.
- Heavy. This form of pathology is characterized by the development of muscle atrophy, hypokalemia, arterial hypertension, and mental disorders.
The course of the disease is also distinguished by the rate of increase of pathological changes - a progressive and torpid form of the disease is isolated. In the first case, the disease develops over 6-12 months. Changes that occur with the patient do not go unnoticed by others. In the torpid course, the signs of the disease are formed in stages, over 3-10 years.
How does appearance change
Recognizing the symptoms of Itsenko-Cushing's disease will not be a big deal even for those who are far from medicine. To do this, you need to know what violations occur with this disease and how the appearance of a person with this ailment changes with them.
Patients suffering from the disease are distinguished by the so-called cushingoid appearance, which is characteristic of:
- specific deposition of adipose tissue in typical areas - on the face, shoulders, neck, chest, back, stomach;
- as the disease progresses, the person acquires a moon shape;
- in the region of VI-VII of the cervical vertebrae, a fatty hump forms;
- the chest and abdomen increase in volume, and the limbs remain relatively thin;
- the skin may peel off, become dry, while a pronounced crimson-marble pattern will be visible on it;
- in the area of the mammary glands, shoulders and lower abdomen, striae and subcutaneous hemorrhage are observed;
- Cushing's patients often suffer from acne and furunculosis.
Other signs and symptoms.
In women with Itsenko-Cushing's syndrome, a malfunction occurs in the menstrual cycle, in some cases, menstruation completely disappears. In addition, one cannot but note the excessive atypical hair growth of the male type - this phenomenon in medicine is called "hirsutism." We are talking about the vegetation on the face, neck, chin, temples, abdomen, chest and intense hair loss on the head.
In men suffering from Cushing's disease, on the contrary, the hair on the body falls out, the growth of stubble on the face stops. Over time, in patients, regardless of gender, libido decreases, sexual desire is lost. If the disease affects children, the delay in sexual development and growth is likely due to a decrease in the secretion of gonadotropic hormone.
Due to calcium reabsorption and the development of osteoporosis, bone deformation occurs, fractures, joint pains are possible. Over time, tachycardia, cardiopathy, and chronic heart failure add to arterial hypertension. Patients in this category are more likely to suffer from bronchitis, pneumonia and tuberculosis.
It is impossible to ignore the defeat of the digestive system in the development of Itsenko-Cushing's syndrome. Patients often complain of increased heartburn, pain in the epigastric region caused by "steroid" peptic ulcer and bleeding within the gastrointestinal tract. Probable violations in the work of the kidneys, lesions of the urinary tract up to uremia are not ruled out.
Neurological problems
The progression of Itsenko-Cushing's pathology threatens the patient with the development of pain, amyotrophic, stem-cerebellar and pyramidal syndromes. But if pain and amyotrophic syndromes are amenable to drug control, then changes caused by stem-cerebellar and pyramidal syndromes cannot be restrained, they are irreversible. Patients may experience ataxia, nystagmus, pathological reflexes, in which the facial and sublingual nerves are involved.
In Itsenko-Cushing's disease, psychoemotional disorders often occur due to neurasthenic, depressive, astheno-dynamic and epileptiform syndromes. A completely natural phenomenon is the deterioration of memory and intelligence, lethargy, and a decrease in the amplitude of emotional fluctuations. Some patients have obsessive suicidal thoughts.
Dog Cushing's Disease
Interestingly, in tetrapods this disease is diagnosed as often as in humans. The cause of the incidence is a similar effect of cortisol on the functioning of the whole organism. In an animal with Itsenko-Cushing's syndrome, immunity, nervous and genitourinary systems are affected, the heart suffers, the condition of the connective tissue and internal organs worsens. The disease almost never develops rapidly, so the pet owners have enough time and good chances to help the animal and prolong its life. The following symptoms should alert:
- increased thirst;
- frequent urination
- exorbitant appetite;
- deterioration of the coat, the appearance of symmetrical bald patches.
Other signs of Cushing's disease can also be observed . In dogs, along with increased urge to urinate, urinary incontinence appears. The pet is gaining excess weight, and fat deposits are localized mainly in the neck, abdomen and chest. Over time, weakened muscles become unable to hold an enlarged abdomen, which makes it sag. The dog becomes inactive, lethargic, drowsy, possibly unbalanced behavior. If the pituitary adenoma was the cause of the disease, coordination disorders are observed.
Examination and Diagnostics
To begin treatment, it is not enough just patient complaints and examination by a doctor. A diagnosis will require consultation by an endocrinologist, neuropathologist, gastroenterologist, cardiologist and gynecologist.
Specialists in their arguments rely on the results of biochemical blood tests - a number of indicators undergo typical changes in Cushing's disease. Diagnostics allows us to conclude that there is an increased content of cholesterol, chlorine and sodium in the blood. In patients with this ailment, blood and urine sugar levels are significantly higher than normal, which indicates the development of a characteristic "steroid" diabetes mellitus.
A blood test for hormones is mandatory, which allows you to identify the concentration of cortisol, adrenocorticotropic hormone, renin. The cortisol content is also taken into account in the patient's urine.
In addition to laboratory research methods, the patient must undergo:
- X-ray of the skull. Particular attention is paid to the study of the Turkish saddle - the location of the pituitary gland. In the x-ray, you can see the macroadenoma.
- X-ray of the spine. It is carried out in order to identify signs of osteoporosis.
- CT and MRI of the brain with the introduction of gadolinium (contrast medium). During diagnostic procedures, macro- and microadenomas are detected.
- Ultrasound of the adrenal gland. In Cushing's disease, bilateral organ hyperplasia is diagnosed. At the same time, an adrenal gland enlargement on one side only is often regarded as the formation of glucose steroids.
Therapy and Surgery
Since Itsenko-Cushing's disease develops against the background of ACTH secretion disorders, treatment involves the elimination of endocrine disorders and the restoration of metabolic processes. The therapeutic program involves the use of medicines, radiotherapy of the hypothalamic-pituitary area. In severe cases, the most effective treatment option will be surgery or a combination of several therapeutic methods.
Medications are prescribed to patients only in the early stages of the disease. Blockade of pituitary functions is an intermediate goal of drug exposure. To achieve it, drugs such as Reserpine, Bromocriptine are prescribed - they actively suppress ACTH secretion. In addition to central-acting hormones, adrenal steroid synthesis blockers can be used in the treatment of Itsenko-Cushing's disease. Be sure to carry out symptomatic therapy, correcting protein, mineral, carbohydrate, electrolyte metabolism.
Radiation therapy is often given to patients who do not have x-ray data on the pituitary tumor. One of the treatment methods is implantation of radioactive isotopes into the pituitary gland. A few months after therapy, a period of remission begins, which is accompanied by a decrease in body weight, blood pressure, stabilization of the menstrual cycle, inhibition of the pathological process and damage to bone and muscle tissue. Irradiation of the pituitary gland is often combined with unilateral removal of the adrenal gland (adrenalectomy).
In severe Cushing's disease, treatment is the removal of adrenal glands from two sides (bilateral adrenalectomy), which further requires lifelong replacement therapy with glucocorticosteroids and mineralocorticoids.
If the cause of the disease is a benign pituitary tumor, endoscopic transnasal or transcranial removal of the adenoma is performed. In some cases, the operation to excise the affected part of the pituitary gland is performed transfenoidally. After removal of the adenoma, remission occurs in a predominant number of cases, but approximately one in five patients develops a relapse.
What is the forecast
Determining the chances of a person recovering with Cushing's disease is not easy. The outcome of the disease depends on the severity of the disease, the age of the patient. With the timely treatment of pathology at an early stage in young patients, a complete recovery is not excluded. While the prolonged course of Cushing's disease leads to persistent changes in the bone tissue, cardiovascular and genitourinary system, negatively affects the ability to work and aggravates the prognosis, even if the underlying cause is subsequently eliminated. The neglected forms of this ailment end fatally in connection with the accession of infections, septic complications, the development of renal failure.
Patients with Itsenko-Cushing pathology should be regularly observed by an endocrinologist, cardiologist, neurologist, gynecologist. In order to prevent the progression of the disease, it is important to avoid exorbitant physical and psychoemotional stress, treat infectious diseases, intoxication in time, and avoid complications. With this diagnosis, work in night shifts is completely ruled out.