How to treat bronze (Addison's) disease. Addison's disease. Symptoms, signs and treatment of Addison's disease The hand turned dark but Addison's disease was ruled out

Content

Hypocorticism or Addison's disease is a disease of the endocrine system, in which the secretion (excretion process) of the hormones of the adrenal cortex decreases. The disease can affect people of all ages, but more often the pathology is diagnosed in women after 20 years. Often the disease is called bronze disease because of the yellow spots that occur on the body.

What is Addison's disease

Bronze disease is a rare disease of the endocrine system, the development of which is based on the destruction of the tissues of the outer layer of the adrenal glands. In this case, there is a deficiency of glucocorticoid hormones (aldosterone, cortisol), which protect the body from stress and are responsible for metabolism, water-salt metabolism. Hypocorticism was first described by British physician Thomas Addison in 1855. The disease disrupts the functioning of the whole organism. If treatment is not started on time, this can lead to serious complications:

• paralysis of the limbs;
• thyrotoxicosis (hyperfunction of the thyroid gland);
• ovarian dysfunction;
• swelling of the brain;
• paresthesia (impaired sensitivity);
• thyroiditis (inflammatory lesion of the thyroid gland);
• anemia (decreased hemoglobin concentration);
• chronic candidiasis (fungal infection).

Reasons for development

Approximately 70% of all cases of Addison's disease are caused by an autoimmune lesion of the adrenal cortex. At the same time, the body's defense system fails and it recognizes the cells of the endocrine glands as foreign. As a result, antibodies are produced that attack the adrenal cortex and damage it. Hypocorticism can cause harmful bacteria, fungi, viruses, immune, genetic disorders. Factors contributing to the occurrence of pathology:

• overweight;
• physical, emotional stress;
• hypofunction (weakening) of the thyroid gland;
• polycystic ovary syndrome;
• allergic reaction;
• prolonged depression;
• hypoglycemia (decrease in glucose levels due to nutritional deficiencies in the diet);
• severe form of diabetes.

Pathology has a primary, secondary form:

1. Reasons for the development of the primary lesion:

• adrenal tuberculosis;
• autoimmune processes;
• hypoplasia (underdevelopment of the organ);
• congenital anomalies;
• surgical removal of the adrenal gland;
• hereditary diseases;
• cancerous tumors, metastases;
• syphilis;
• AIDS;
• fungal diseases;
• decreased sensitivity of the adrenal glands.

1. The secondary form of the disease may appear due to:

• mechanical injury;
• meningitis (inflammation of the membranes of the brain);
• ischemia (reduced blood supply);
• benign, malignant tumor;
• brain irradiation.

If, after confirmation of bronze disease and a full course of treatment, the patient refuses further use of drugs, adrenal iatrogenic insufficiency may occur. This condition provokes a rapid drop in the level of endogenous glucocorticoids against the background of prolonged replacement therapy and inhibition of the activity of one's own glands.

Symptoms

The clinical picture of Addison's disease develops slowly. Symptoms for many years may be mild and manifest themselves only when an Addisonian crisis occurs. This is a life-threatening condition in which the level of glucose drops sharply. As a rule, important symptom complexes of pathology are:

• skin pigmentation;
• asthenia and adynamia;
• disorders of the gastrointestinal tract (gastrointestinal tract);
• hypotension (lowering blood pressure).

In addition, Addison's disease has the following manifestations:

• darkening of the skin;
• muscle weakness;
• weight loss, loss of appetite;
• cravings for sour, salty foods;
• increased thirst;
• stomach ache;
• vomiting, nausea, diarrhea;
• dysphagia (impaired swallowing);
• tremor (trembling) of the hands;
• dehydration;
• tachycardia (rapid heartbeat);
• irritability, irascibility, depression;
• impotence in men, amenorrhea (absence of menstruation) in women;
• dizziness;
• decrease in working capacity;
• the appearance of yellow spots;
• drying of mucous membranes, skin;
• convulsions.

Asthenia and adynamia

Physical, mental fatigue (asthenia, adynamia) and impotence are considered the earliest and permanent symptoms of bronze disease. Often the onset of the disease cannot be precisely determined. At the very beginning of the development of pathology, rapid fatigue appears from work that was previously performed without serious labor costs. In addition, there is a general weakness that develops with a normal lifestyle. Such sensations progress, intensify and lead the patient to a state of complete physical impotence.

Skin pigmentation

Violation of the uniform color of the skin or the appearance of pigmentation is the most important, conspicuous symptom. Abundant deposition of melanin (a pigment that does not contain iron) in the cells of the malpighian layer of the epidermis contributes to the appearance of brown, bronze or smoky spots on the skin. Starting on the face, pigmentation can capture the entire surface of the skin or be localized in certain places: forehead, interphalangeal joints, neck, nipples, scrotum, around the navel, etc.

Sometimes large dark spots appear on the face. The bluish color stains the lips, nipples, mucous membranes of the vagina and intestines in women. Along with a strong violation of pigmentation, the patient has skin areas that are poorly pigmented or even completely devoid of melanin, which stand out sharply against the background of the surrounding dark skin - the so-called vitiligo, or leukoderma. In the primary form of Addison's disease, this symptom appears one of the first, while it can be several years ahead of other signs of the disease.

Addison syndrome in women

When Addison's disease occurs in women, there is a decrease in muscle mass due to loss of appetite. In addition, the patient gradually loses hair on the pubis and in the armpits, and the skin loses its elasticity. The lack of estrogen and testosterone leads to the cessation of monthly menstruation, a decrease in libido. Other distinctive features of the course of the disease in women:

• Gastrointestinal disorders (nausea, diarrhea, vomiting) are noted. A stomach ulcer, gastritis develops, because. glucocorticoids no longer protect the intestinal mucosa from the effects of aggressive factors.
• Pigmentation of the skin of large folds and open areas is characteristic. Dark spots may appear on the inside of the cheeks and gums.
• The risk of gynecological diseases (mastopathy, fibroids, endometriosis) increases.
• The water-salt metabolism in the body is disturbed, as a result of which dehydration occurs, the skin becomes dry, cavities appear on the cheeks, fainting occurs, and blood pressure (blood pressure) decreases.
• The brain and nervous system also suffer from dehydration, which is manifested by numbness of the limbs, sensory disturbances, and muscle weakness.
• Reproductive abilities are reduced up to miscarriage, infertility.

Treatment

Only an endocrinologist can establish an accurate diagnosis for Addison's disease based on the results of the diagnosis. To distinguish hypocorticism from Conn's disease, Itsenko-Cushing's, the specialist prescribes the patient to do a biochemical analysis of blood, urine, and an x-ray of the skull. Magnetic resonance and computed tomography are performed to detect signs of adrenal tuberculosis. The most important diagnostic method that confirms the disease is the electrocardiogram. After all, a violation of the water-salt balance often negatively affects the work of the heart.

The main treatment for hypocorticism is lifelong hormonal replacement therapy, i.e. taking glucocorticosteroid drugs - analogues of cortisol and aldosterone (Cortisone). The specialist selects the dosage individually. The duration of treatment depends on the form and severity of the disease. To prevent the occurrence of an Addisonian crisis against the background of an injury, an infectious disease, an upcoming operation, the dosage of hormonal agents should be reviewed by a doctor. How to treat:

1. The intake of glucocorticosteroids begins with physiological doses, then the amount of the drug used is gradually increased until the level of hormonal levels is normalized.
2. After 2 months of hormone treatment, a control blood test is performed to assess the effectiveness of therapy. If necessary, the dosage of drugs is adjusted.
3. With the tuberculous nature of the disease, therapy is carried out with the help of Rifampicin, Streptomycin, Isoniazid. The patient's condition is assessed by a phthisiatrician and an endocrinologist.
4. Compliance with a special diet. It is necessary to exclude from the diet: bananas, nuts, peas, coffee, potatoes, mushrooms. It is useful to eat liver, carrots, eggs, beans, legumes, fresh fruits. You should eat in small portions 6 times a day.

Video

Addison's disease has another name - bronze disease. By this is meant a violation of the functioning of the adrenal glands. In turn, this disrupts the hormonal balance, as a result, the synthesis of glucocorticoids decreases or completely disappears.

Addison-Birmer disease has a large number of symptoms, which are mainly due to the defeat of most of the cortex. The cause of this disease can be different. In 8 out of 10 cases, Addison-Birmer disease develops due to an autoimmune process in the body.

But also sometimes the disease can be accompanied by tuberculosis, which has affected the adrenal glands. Pathology can be congenital and can be inherited. The autoimmune type of the disease is most common in the female half of the population.

The most common symptoms of Addison's disease are soreness, gastrointestinal disturbances, and hypotension. Pathology can lead to metabolic disorders. Such a disease can also be treated with the help of traditional medicine, which will enhance the work of the adrenal glands, as well as help in the fight against microbes and inflammation.

General characteristics of the disease

Addison's disease, the photo of which clearly shows the affected area, can be with both primary and secondary insufficiency. As many people know, pathology affects the endocrine glands responsible for the production of some of the most important hormones in the human body. These organs have 2 zones:

• crust;
• brain matter.

Each zone is responsible for synthesizing different types of hormones. The medulla produces norepinephrine and adrenaline. They are especially necessary for a person in a stressful situation, these hormones will help to use all the reserves of the body.

Other hormones are also synthesized in the cortex.

• Corticosterone. It is necessary for the balance of water and salt metabolism in the body, and is also responsible for the regulation of electrolytes in blood cells.
• Deoxycorticosterone. Its synthesis is also required for water-salt metabolism, in addition, it affects the efficiency and duration of muscle use.
• Cortisol is responsible for the regulation of carbon metabolism, as well as for the production of energy resources.

The pituitary gland, a gland located in the region of the brain, has a great influence on the adrenal cortex. The pituitary gland produces a special hormone that stimulates the adrenal glands.

As mentioned above, Addison-Birmer disease has two types. Primary this is the disease itself, when the work of the adrenal glands is completely disrupted due to negative factors. The secondary implies a decrease in the amount of ACTH synthesized, which, in turn, impairs the functioning of the endocrine glands. In a situation where the pituitary gland produces an insufficient amount of hormones for a long period, dystrophic processes may begin to develop in the adrenal cortex.

Causes of the disease

The primary form of Addison-Birmer disease is quite rare. It is equally likely to be found in both men and women. In most cases, the diagnosis is made to people whose age is from 30 to 50.

There is also a chronic form of the disease. Such a development of pathology is possible with various negative processes. In almost all cases, namely in 80%, the cause of Addison-Birmer disease is an autoimmune condition of the body. In 1 out of 10 cases, the cause of the pathology is the defeat of the adrenal cortex by an infectious disease, for example, tuberculosis.

For the remaining 10% of patients, the causes may be of a different nature:

• this can be affected by long-term use of drugs, in particular, glucocorticoids;
• types of fungal infection;
• injury to the endocrine glands;
• amyloidosis;
• tumors of both benign and malignant nature;
• bacterial infections with a weakened human immune system;
• pituitary dysfunction;
• genetic predisposition to the disease.

Other syndromes can also develop due to Addison's disease, such as an adrenal crisis, which occurs when the concentration of adrenal hormones is too low.

There are the most likely causes of the crisis:

• severe stressful condition;
• violations in the dosage when compiling a course of treatment with hormonal drugs;
• an infectious lesion of the adrenal cortex can aggravate the disease;
• trauma to the adrenal glands;
• circulatory disorders, such as blood clots.

Symptoms of the disease

Symptoms of Addison's disease directly depend on the violation of the synthesis of certain types of hormones. Clinical manifestations of the disease may be different. The determining factors are the form of pathology and its duration.

The most common clinical manifestations of pathology are as follows:

• Addisonian pathology has its own name of bronze disease for a reason. The most obvious sign of this disease is a violation of pigmentation. The skin changes its color. The color of mucous membranes changes. It's all about too much pigmentation. With a lack of adrenal hormones, much more ACTH is produced, this is explained by the need to stimulate the work of the endocrine glands.
• One of the common clinical manifestations of the disease is chronic hypotension. This can lead to dizziness and fainting, increased sensitivity to low temperatures.
• With insufficient work of the endocrine glands, the whole organism weakens. If you have constant fatigue, rapid fatigue, you should consult a medical specialist.
• With this pathology, there are often disturbances in the work of the gastrointestinal tract, this can manifest itself in the form of vomiting, constant nausea and diarrhea.

• The disease can affect the emotional component. Depression is one of the clinical manifestations of Addison's disease.
• Patients reported increased sensitivity to stimuli. The sense of smell, hearing is aggravated, the person feels the taste of food better. In most cases, patients prefer to eat salty foods.
• Pain in the muscle tissue can also be a symptom of Addisonian pathology. This is explained by an increase in the concentration of potassium in the blood vessels.
• As mentioned above, one of the clinical manifestations of the disease is an adrenal crisis, which occurs as a result of a sharp decrease in the level of hormones of the endocrine glands. The most popular symptoms of a crisis are pain in the abdomen, low blood pressure, and disturbed salt balance.

Diagnosis of the disease

First of all, patients pay attention to changes in the shade of the skin. This phenomenon signals an insufficient activity of adrenal hormones. When referring to a medical specialist in this situation, he determines the ability of the adrenal glands to increase the synthesis of hormones.

Diagnosis of Addison's disease occurs by the introduction of ACTH and measurements of the content of cortisol in the blood vessels before the administration of the drug and 30 minutes after vaccination. If the potential patient does not have problems with the functions of the adrenal glands, the level of cortisol will increase. If the concentration of the test substance has not changed, the person has disturbances in the functioning of the endocrine glands. In some cases, for a more accurate diagnosis, the content of the hormone in urea is measured.

Treatment of pathology

In the course of treatment, special attention should be paid to the diet. It should be varied, it should contain the necessary amount of proteins, fats and carbohydrates to provide the body. Particular attention should be paid to vitamins B and C. They can be found in bran, wheat, fruits and vegetables. In addition, the patient is advised to drink more decoctions based on wild rose or black currant.

With Addison's disease, the sodium content in the body decreases, for this reason it is recommended to focus on salty foods. In addition, the pathology is characterized by an increased concentration of potassium in the blood vessels, it is recommended not to include foods that are rich in potassium in the diet. These include potatoes and nuts. Patients are advised to eat as often as possible. Before going to bed, medical experts recommend having dinner, this will reduce the chance of hypoglycemia in the morning.

Almost all folk recipes are aimed at stimulating the adrenal cortex. Traditional medicine has a mild effect, side effects are practically absent. The use of folk recipes will not only improve the functioning of the adrenal glands, but also have a positive effect on the state of the whole organism as a whole. With the help of this approach, it is possible to normalize the work of the gastrointestinal tract, to counteract chronic inflammatory processes. It is recommended to use several recipes in turn, this will avoid the addiction of the body.

Prevention and forecasting

If therapy was started in a timely manner and all the recommendations of a medical specialist were followed, the outcome of the disease will be favorable. The disease will not affect life expectancy in any way. In some cases, Addison's disease is accompanied by a complication - an adrenal crisis. In such a situation, you should immediately seek the advice of a medical specialist. A crisis can be fatal. Addison's disease is accompanied by rapid fatigue, weight loss and loss of appetite.

Changes in the shade of the skin do not occur in all cases, deterioration in the work of the endocrine glands occurs gradually, so it is difficult for a person to detect this on their own. In such a situation, a critical condition develops abruptly and unexpectedly for the patient. Most often, the cause is some negative factor, such as stress, infection or injury.

Since Addison's disease is often autoimmune in nature, there are practically no preventive measures. You should monitor your immune system, avoid drinking alcohol, smoking. Medical experts recommend timely attention to the manifestations of infectious diseases, especially tuberculosis.

The adrenal cortex produces vital hormones - glucocorticoids and mineralocorticoids. In addition, a small amount of sex hormones is also produced in this endocrine organ.

If the cortex is affected by any pathological process, then the patient develops a serious disease - primary adrenal insufficiency (Addison's disease). This chronic pathology has many consequences for the whole organism and even threatens life.

The prevalence of the disease is low. There are 40-60 cases of pathology per 100 thousand of the population. Women suffer from Addison's disease much more often. Especially this difference in the structure of morbidity by sex of patients has become noticeable recently.

Why does the disease occur

The cause of Addison's disease in most cases is autoimmune inflammation. Antibodies to the body's own cells produce defense systems. The immune system mistakes the adrenal cortex for foreign tissue and begins to destroy it. When most of the endocrine cells have already died, the first symptoms of bronze disease appear.

What can provoke an autoimmune aggression? Physicians cannot unambiguously answer this question. Probably, the pathology of the body's defenses is associated with heredity, genetic mutations, excessive insolation (sunlight, solarium), and adverse environmental conditions.

The autoimmune process is responsible for 80–90% of cases of the disease. The destruction of the adrenal cortex can occur for other reasons. So, in adverse social conditions, the disease provokes tuberculosis. To date, this infection is the cause of about 10% of all cases of chronic adrenal insufficiency.

Less commonly, the cause of the disease is trauma, surgical destruction, thrombosis, benign or malignant tumor.

Whatever caused primary adrenal insufficiency, its clinical picture and principles of treatment differ slightly.

Signs of illness

Symptoms of the disease are associated with insufficiency of glucocorticoids and mineralocorticoids. Some of the symptoms appear due to the excessive stimulating effect of the pituitary gland. Endocrine cells in the brain try to increase the work of the adrenal cortex by releasing large amounts of tropic hormones (primarily adrenocorticotropin). Melanostimulating hormone is also synthesized in the pituitary gland. With Addison's disease, too much of it is released and because of this, hyperpigmentation of the skin and mucous membranes appears. The dark coloration can be so pronounced that it has become the source of another name for the disease - "bronze" disease (see photo). The first signs of hyperpigmentation appear in places where the skin rubs against clothes, in natural folds and at the site of surgical sutures or scars.

A patient with Addison's disease is troubled by severe general weakness. Fatigue can be so severe that it is difficult for the patient to get out of bed, take a few steps, or just sit.

The disease is always accompanied by hypotension. Blood pressure during the day is almost always below 90/60 mm Hg. Art. If the patient gets up abruptly, then hypotension increases. When changing the position of the body, there may be episodes of dizziness, darkening in the eyes, loss of consciousness.

Primary insufficiency of the adrenal cortex leads to disruption of the digestive tract. The patient loses his appetite. Body weight begins to decrease rapidly. There are changes in food habits. There may be cravings for salt.

Disorders of the digestive system are manifested by nausea and vomiting, diarrhea. These phenomena contribute to the rapid depletion of the body.

The emotional state of patients is usually depressed. Specialists often diagnose depression and other disorders. In severe cases, psychosis (delusions, hallucinations, aggressive behavior) may develop.

Addisonian crisis

Addisonian crisis - this condition develops due to an acute insufficiency of cortical hormones. Without treatment, the crisis leads to the death of the patient.

Signs of a sharp deterioration in the condition:

• severe muscle pain;
• vomit;
• a sharp drop in blood pressure;
• psychosis;
• loss of consciousness;
• convulsions.

Blood tests reveal a sharp drop in glucose and sodium, an increase in the concentration of calcium, potassium and phosphorus.

How is the diagnosis established?

Examination for suspected primary adrenal insufficiency is carried out by an endocrinologist. Diagnosis of Addison's disease includes a standard or short test with adrenocorticotropin. Additionally, the patient is prescribed a blood test for cortisol, renin, blood sugar, electrolytes, adrenocorticotropin and a complete blood count.

The disease is characterized by:

• there is not a sufficient increase in cortisol levels during trials;
• blood cortisol is reduced;
• renin and ACTH are elevated;
• hyponatremia;
• hyperkalemia;
• an increase in neutrophils and eosinophils in the general blood test;
• hyperglycemia (increased blood sugar).

Medical tactics

Treatment of chronic adrenal insufficiency requires hormone replacement therapy. This tactic involves the appointment of glucocorticoids and mineralocorticoids on an ongoing basis.

Usually the patient receives medicines in the form of tablets. 60-70% of the daily dose is prescribed before breakfast, the rest - before lunch.

If there is a risk of deterioration of health (crisis), then the dose of drugs is increased by 50-100%. Such measures are needed for acute diseases, operations, injuries, etc.

If the patient develops a sharp drop in blood pressure, then hormones begin to be injected (hydrocortisone).

Addisonian crisis is treated with simultaneous administration of hydrocortisone, sodium chloride and glucose.

We explore the causes and symptoms that manifest Addison's disease. We will also study how the diagnosis is carried out and what medications are used for treatment, which can increase the duration of a normal life.

What is Addison's disease

Addison's Disease is metabolic pathology, with which adrenal glands produce hormones in insufficient quantities .

In this condition, the secretion of the adrenal glands is unable to meet the physiological needs of the body, resulting in acute symptoms, which, if left untreated, can threaten the life of the patient.

The disease can affect anyone at any age, but usually occurs around 40 years of age. It is assumed that the incidence on a global scale is 1 case for every 100,000 people.

From what has been said, it is clear that:

• decreased production of aldosterone causes an imbalance of water, sodium and potassium in the body, which is determined by the expulsion of sodium and the retention of potassium. This condition can lead to hypotension (low blood volume) and dehydration;
• cortisol deficiency causes metabolic disorders and hypoglycemia problems, which makes the body weak.
• decrease in androgens can lead to decreased desire, hair loss and loss of sense of well-being due to mental problems.

Symptoms of low adrenal hormone levels

Symptoms of the disease depend on the level of concentration of hormones of the adrenal cortex in the blood. The decrease in concentration, in most cases, is associated with the destruction of adrenal cells. Destruction, as a rule, develops slowly.

Thus, the clinical picture of Addison's disease in the initial stage is characterized by very mild and non-specific symptoms, which slowly progress as destructive lesions of the gland spread.

When the damage covers 90% of the secreting cells, chronic adrenal insufficiency develops.

Summarizing the main symptoms of Addison's disease, the following should be noted:

• Asthenia. Decreased muscle strength to the point where it is difficult to perform daily activities. Fatigue is present even at rest or after an absolutely insignificant effort.
• hypotension. Accompanied by dizziness, and in severe cases even fainting and falls. Decreased blood pressure, which decreases further when standing upright, is a direct result of low aldosterone levels. This hormone blocks the excretion of sodium in the urine. Low levels of aldosterone lead to loss of sodium and fluid, resulting in a decrease in blood volume and blood pressure.
• hypoglycemia. Decreased blood sugar levels as a result of low cortisol levels. A decrease in the concentration of the latter, in fact, reduces gluconeogenesis, namely the production of glucose from proteins and lipids, causing hypoglycemia. This is exacerbated by hypotension and asthenia.
• Dehydration. It is caused by excessive diuresis caused by the loss of sodium salts in the urine. Accompanied by an irresistible craving for salty foods.
• Weight loss and anorexia. This is a consequence of hypoglycemia and impressive fluid loss in the urine.
• Skin hyperpigmentation. Pigmentation can be observed both on the skin exposed to the sun and on hidden areas of the body that are usually not accessible to sunlight: mucous membranes of the mouth, gums, skin folds, scars, etc. All this comes from the fact that the pituitary gland stimulates the adrenal glands to produce more corticotropin, which, in turn, stimulates the production of melanin, which is responsible for skin pigmentation.
• Gastrointestinal. That is, nausea, vomiting, diarrhea, abdominal cramps.
• pain in joints and muscles.
• Migraine.
• Mood related problems: irritability, excessive anxiety, bad mood and melancholy, and in severe cases depression. All these problems are associated with reduced secretion of androgens, which act on the psyche, providing a sense of general well-being.
• Loss of body hair. The symptom is also associated with low androgen levels.

In a small number of cases, the symptoms of the disease, instead of the chronic form, develop quickly and suddenly, this state is called acute adrenal insufficiency or Addison crisis. This situation requires immediate medical attention and proper patient care.

symptomatology, which characterizes the Addison crisis, consists of the following:

• Sharp and excruciating pains that cover the abdomen, back, lower part or legs.
• Fever and confusion, trouble making meaningful conversations.
• Vomiting and diarrhea with danger of dehydration.
• Very low blood pressure.
• Loss of consciousness and inability to stand.
• Very low blood glucose.
• Very low sodium levels in the blood.
• High levels of potassium in the blood.
• High levels of calcium in the blood.
• Involuntary muscle contractions.

Causes of Addison's disease - primary and secondary

The hormonal deficiency that is characteristic of Addison's disease can be caused by problems originating either from the adrenal glands (primary adrenal insufficiency) or from other sources (secondary adrenal insufficiency).

When primary adrenal insufficiency a decrease in hormone levels is a consequence of the destruction of glandular tissue cells, which, in turn, can be caused by:

• immune system error. Which, for unknown reasons, recognizes the cells of the adrenal cortex as foreign and destroys them, leading to the development of pathology.
• Granulomas from tuberculosis. In this case, granulomas are the result of an inflammatory process caused by a viral infection.
• Fungal infections or cytomegalovirus infections. They occur in patients suffering from immunosuppressive disorders.
• Primary or metastatic adrenal tumors(most often lymphoma or lung cancer).
• Bleeding from the adrenal glands. An example of such a disease is the Waterhouse-Frideriksen syndrome. This is severe bleeding resulting from a severe bacterial infection, usually caused by Neisseria meningitidis meningococci.
• adrenal infarction. Necrosis of the connective tissue of the gland due to insufficient blood supply.
• genetic mutations that affect the functioning of the adrenal glands. They are quite rare.

When secondary adrenal insufficiency the adrenal glands remain functional and efficient, and low hormone levels are the result of:

• Problems with the pituitary gland. It is responsible for the secretion of adrenocorticotropic hormone, which has the task of stimulating the adrenal cortex.
• Stopping corticosteroid therapy. Such therapy is often carried out in the case of patients suffering from asthma and rheumatoid arthritis. A high level of corticosteroids reduces the level of adrenocorticotropic hormone in the blood. A decrease in the latter leads to a sharp decrease in the stimulation of adrenal cells, which ultimately causes a kind of atrophy. Restoring their normal operation may take a considerable time (about 6 months). During this period, the patient may have an Addison's crisis.
• Violation of steroid biosynthesis. To produce cortisol, the adrenal glands must receive cholesterol, which is converted through appropriate biochemical processes. Therefore, in any situation where the flow of cholesterol to the adrenal glands stops, a state of cortisol deficiency develops. Such situations, although very rare, occur with Smith-Lemli-Opitz syndrome, abetalipoproteinemia syndrome, and certain medications (eg, ketoconazole).

Diagnosis of adrenal insufficiency

The doctor makes the first assumption of the diagnosis based on the patient's history and a detailed analysis of symptoms and signs. This hypothesis is then tested by a series of surveys and tests.

Blood analysis, which should include:

• Low levels of cortisol.
• High levels of ACTH.
• Low cortisol response to ACTH stimulation.
• The presence of specific antibodies to the adrenal system.
• The sodium level is below 130.
• Potassium level over 5.

Measuring Glucose After Insulin Injection. Insulin is administered to the patient and blood sugar and cortisol levels are measured at regular intervals. If the patient is healthy, blood sugar levels decrease and cortisol levels increase.

Stimulation of the adrenal glands by ACTH. The patient is injected with synthetic adrenocorticotropic hormone, and then the cortisol concentration is measured. Low levels indicate adrenal insufficiency.

CT scan abdominal cavity. Determines the size and any anomalies of the glands.

Magnetic resonance imaging of the Turkish saddle(the region of the skull where the pituitary gland is located). Identifies possible malfunctions in the pituitary gland.

Therapy for Addison's disease - restore hormone levels

Regardless of the cause, Addison's disease can be deadly, so the hormones that the adrenal glands produce in insufficient quantities must be supplied to the body.

Treatment of the disease involves the introduction of:

• Corticosteroids. In particular, fludrocortisone, to compensate for the deficiency of aldosterone and cortisone.
• Androgens. The introduction in sufficient quantity improves the feeling of well-being, and therefore improves the quality of life.
• Sodium supplements. Serve to reduce fluid loss in the urine and increase blood pressure.

When Addison crisis immediate treatment is needed because it is very life-threatening for the patient.

Treatment includes:

• Hydrocortisone.
• Saline infusions to increase blood volume.
• The introduction of glucose. To increase blood sugar levels.

Due to the danger of a sudden Addison's crisis, patients suffering from the disease should wear emergency medical bracelet. In this way, the medical staff will be immediately notified of the alarm.

Life expectancy in Addison's disease

Correctly treated Addison's disease life expectancy is normal. The only possible danger may come from the Addisonian crisis. Therefore, patients are advised to wear an information bracelet, as well as a cortisol injection syringe in case of emergency.

Alexandra Warshal about the syndrome, describing which, Thomas Addison became the "father of endocrinology"

In 1849, Thomas Addison described primary chronic adrenal insufficiency (otherwise, bronze disease) and identified the main signs of the disease: "lethargy and weakness, palpitations, abdominal pain and discoloration of the skin."

Prevalence

Primary adrenal insufficiency is rare: according to Russian authors, in 1 in 4000-6000 hospitalized patients. American endocrinologists provide data on 39-60 cases of adrenal insufficiency per 1 million population. Chronic adrenal insufficiency (CAN) is more common in men; the ratio of men and women suffering from this disease is 2:1. According to German doctors - Oelkers and his colleagues - the average age at which the disease is diagnosed is 40 years (from 17 to 72).

Etiology and pathogenesis

Clinical manifestations of CNN occur when the functional tissue of the adrenal cortex is 90% impaired by the pathological process. Occasionally, this occurs with bilateral metastases of carcinoma of the lungs, mammary glands and intestines, cytomegalovirus adrenalitis in HIV-infected people or with HIV adrenalitis (which develops in 5% of patients in the late stages of the disease on the background of opportunistic infections) with antiphospholipid syndrome.

The main causes of chronic adrenal insufficiency are autoimmune adrenalitis (60-65% of cases); tuberculosis infection; deep mycoses, amyloidosis, histoplasmosis, hemochromatosis (10% of cases).

In autoimmune adrenalitis, there is intense lymphoid infiltration of the adrenal cortex and proliferation of fibrous tissue with severe atrophy of functional cells. In the blood serum of such patients, antibodies to microsomal and mitochondrial antigens of the cells of the adrenal cortex are found. Like other autoimmune diseases, this lesion is more common in women. Autoimmune adrenalitis is often a component of autoimmune polyglandular syndrome type I and II.

Autoimmune polyglandular syndrome type I develops in childhood (about 10-12 years of age) and includes hypoparathyroidism, adrenal insufficiency, and candidiasis. Often associated with hypogonadism, pernicious anemia, alopecia, vitiligo, and chronic active hepatitis. Autoimmune polyglandular syndrome type II occurs in adults and is characterized by the triad of diabetes mellitus, autoimmune thyroid disease, and adrenal insufficiency.

With tuberculous lesions, the adrenal glands can be enlarged, but more often wrinkled, fibrously changed. The adrenal medulla (synthesizing adrenaline and norepinephrine), which is almost always completely rarefied, is also involved in the pathological process. Active tuberculous process in the adrenal glands is extremely rare. As a rule, tuberculosis infection spreads to the adrenal glands hematogenously from foci localized in the lungs, bones, genitourinary system and other organs.

In primary chronic renal failure, the amount of secreted mineralocorticoids and glucocorticoids decreases and, according to the negative feedback system, the secretion of ACTH and the β-melanocyte-stimulating hormone associated with its secretion increases, which causes hyperpigmentation in Addison's syndrome.

Glucocorticoids (cortisol) are synthesized in the zona fasciculata of the adrenal cortex under the influence of ACTH and are insulin antagonists. They increase blood glucose levels, increase gluconeogenesis from amino acids in the liver, inhibit the uptake and utilization of glucose by peripheral tissue cells, increase glycogen synthesis in the liver and skeletal muscles, increase protein catabolism and reduce their synthesis, increase fat catabolism in subcutaneous adipose tissue and other tissues. . Glucocorticoids also have a certain mineralocorticoid effect.

Symptoms of Addison's Syndrome

Most symptoms of Addison's disease are relatively nonspecific. Almost all patients complain of weakness, fatigue and weight loss. Orthostatic hypotension, arthralgias, myalgias, and an increased craving for salt may also occur. In some cases, gastrointestinal symptoms may be predominant and, as a result, make it difficult to detect adrenal insufficiency. Mental symptoms range from mild memory impairment to overt psychosis, so some patients are misdiagnosed with depression or anorexia nervosa.

Patients, as a rule, cannot determine the time of onset of the disease and indicate a constantly progressive general and muscular weakness, intensifying by the end of the day, in contrast to patients with neurasthenia, in whom general weakness decreases in the evening. As adrenal insufficiency progresses, weakness turns into adynamia, speech slows down, the voice becomes quiet. Often, unusual weakness is detected during intercurrent infections or during a period of dysfunction of the gastrointestinal tract. Muscle weakness develops as a result of a violation of carbohydrate and electrolyte metabolism. Along with general weakness, there is weight loss. These two symptoms are present in all patients with CNN. Weight loss occurs due to dehydration, decreased appetite and the subsequent addition of nausea and vomiting.

Mineralocorticoids (aldosterone, deoxycorticosterone) are synthesized in the glomerular zone of the adrenal cortex under the influence of angiotensin II, cause increased tubular reabsorption of sodium and chloride ions, water, and at the same time increase tubular potassium excretion and increase the hydrophilicity of tissues, promote the transfer of fluid and sodium from the vascular bed to the tissues. Mineralocorticoids increase blood volume and increase blood pressure.

hyperpigmentation seen in 90% of patients. The deposition of melanin primarily increases in areas of skin friction, in areas exposed to sunlight, the nipples of the mammary glands, as well as on the mucous membranes (lips, cheeks, etc.). In the future, generalized hyperpigmentation develops, associated with an excess secretion of ACTH and β-melanocyte-stimulating hormone. Often fresh scars are pigmented, the number of freckles increases. Some patients, against the background of general hyperpigmentation of the skin, have areas of depigmentation - vitiligo, which serves as a marker of an autoimmune process.

Arterial hypotension detected in 88-90% of patients. Systolic blood pressure 90 or 80 mm Hg, diastolic - below 60 mm Hg. In rare cases, diastolic pressure may be normal. A decrease in plasma volume leads to a decrease in cardiac output and stroke volume. The pulse is soft, small, slow. Dehydration and a decrease in the total amount of sodium in the body lead to a decrease in extracellular fluid volume and are one of the factors of hypotension. Another factor is a reduced tone of the vascular wall due to a decrease in the level of cortisol and catecholamines.

Notable feature - ear cartilage calcification May accompany long-term adrenal insufficiency of any origin.

The functions of the gastrointestinal tract are impaired. The most common nausea, vomiting, anorexia, constipation, followed by diarrhea. The secretion of hydrochloric acid and pepsin decreases in the stomach. The pathogenesis of gastrointestinal symptoms is associated with increased secretion of sodium chloride into the intestinal lumen. Vomiting and diarrhea increase sodium loss and lead to acute adrenal insufficiency. Patients with primary chronic renal failure have an increased need for salt.

hypoglycemia develops as a result of a decrease in the secretion of cortisol (contrinsular hormone), a decrease in gluconeogenesis, and glycogen stores in the liver. Attacks of hypoglycemia develop in the morning (on an empty stomach) or after a long break between meals and are accompanied by weakness, irritability, hunger, sweating.

nocturia is one of the common symptoms of HNN.

Change in CNS function manifests itself in a decrease in mental activity and memory, concentration of attention, sometimes states of depression and acute psychosis. Replacement therapy normalizes the function of the central nervous system and the listed symptoms decrease in direct proportion to the normalization of the level of cortisol in the blood. In women suffering from CNN, hair loss (pubic hair, in the armpits) is noted due to the fact that their adrenal glands are the main site for the synthesis of androgens (in men, they are synthesized mainly by the testicles).

Patients with Addison's syndrome may have decreased libido and potency women may have amenorrhea.

Lab Finds

The most common violations in the blood test are an increase in the level of potassium (above 5 mmol / l) and creatinine with a decrease in the level of sodium (up to 110 mmol / l) and chlorine (below 98.4 mmol / l). Serum calcium is rarely elevated. Hypercalcemia in such cases is combined with hypercalciuria, thirst, polyuria and hypostenuria. Patients may also develop normocytic normochromic anemia, and peripheral blood smears show eosinophilia and relative lymphocytosis. Often fix a slight increase in the content of TSH (usually< 15 мкЕд/мл). Остается неясным, обусловлено ли это повышение ТТГ сопутствующим аутоиммунным заболеванием щитовидной железы, отсутствием подавления ТТГ эндогенными стероидами или развитием эутиреоидного патологического синдрома.

Renal dysfunctions are observed: the glomerular filtration rate and renal blood flow decrease.

Metabolic disorders and electrolyte imbalance lead to changes in the ECG. An enlarged and pointed T wave is usually found, which in some leads may even exceed the QRS complex in height. Slowing of atrioventricular or intraventricular conduction is possible.

The diagnosis is based not only on the data of the clinical picture and laboratory tests, but also on direct confirmation of a decrease in the functional activity of the adrenal glands. If the level of corticosteroids in the blood taken at 8-10 am is less than 170 nmol / l (6 μg / 100 ml), then the diagnosis of adrenal insufficiency is not in doubt. The presence or absence of pigmentation indicates the primary or secondary nature of the disease. In primary CNN, ACTH levels are usually elevated, while in secondary CNN, they are lower. Also, to clarify the diagnosis, a number of pharmacodynamic tests are performed - fluctuations in cortisol are recorded with the introduction of ACTH or insulin. The test with insulin makes it possible to differentiate the tuberculous lesion of the adrenal cortex from its destruction by an autoimmune process. Tuberculous lesions are accompanied by destruction of the medulla (in which catecholamines are synthesized), while in lesions of the autoimmune process, changes occur only in the cortical layer of the adrenal gland. Therefore, by determining the content of adrenaline in the blood serum during the period of the insulin test, along with glucocorticoids, it is possible to establish the cause that caused chronic adrenal insufficiency.

For the diagnosis of hypoaldosteronism, the concentration of aldosterone in the blood plasma or its excretion in the urine is determined. And here preference should be given to pharmacodynamic tests. Angiotensin is a specific stimulator of aldosterone secretion. If the aldosterone concentration does not rise at the end of the angiotensin infusion, this indicates hypoaldosteronism.

Treatment

How to treat Addison's syndrome? Patients with Addison's syndrome require constant corticosteroids. In most cases, the introduction of only glucocorticoids is sufficient for complete compensation; sometimes additional appointment and mineralocorticoids is required. Hydrocortisone (cortisol) is the drug of choice and is given at 30 mg daily (15–20 mg in the early morning and 5–10 mg at noon). Cortisone is usually used in a daily dose of 40-50 mg. Other synthetic glucocorticoids (prednisolone, dexamethasone, triamcinolone, etc.) are less desirable because they do not have a mineralocorticoid effect. In case of severe mineralocorticoid insufficiency, DOXA (5 mg 1 time per day intramuscularly), deoxycorticosterone trimethylacetate (1 ml of a 2.5% solution parenterally 1 time in 2-3 weeks) or fluorohydrocortisone / cortinef (0.05-0 .1 mg per day).

An excess of mineralocorticoids is fraught with edema, headache, high blood pressure, hypokalemic alkalosis, and muscle weakness. In these cases, it is necessary to cancel mineralocorticoids and prescribe potassium chloride.

In women with CNN, pregnancy and normal delivery are possible. As a rule, during pregnancy, the need for mineralocorticoids decreases due to increased secretion of progesterone. However, the intake of glucocorticoids must be increased, and in some cases parenteral administration of hydrocortisone is required. During childbirth, glucocorticoids are administered intravenously.

In infectious diseases of mild or moderate severity, the dose of glucocorticoids is doubled or tripled. If the disease occurs with vomiting, as well as with the appearance of symptoms of an adrenal crisis, intensive care of the patient in a hospital is necessary. Surgical interventions in patients with CNN are performed under the condition of intravenous administration of hydrocortisone (100-200 mg, depending on the type of operation). In the postoperative period, loading doses of glucocorticoids are reduced quickly - 2-3 days after the elimination of the stressful situation.

Forecast

Before the use of glucocorticoids, life expectancy in patients with adrenal insufficiency was less than 6 months. Today, with timely diagnosis and adequate replacement therapy, in patients with autoimmune adrenalitis, life expectancy does not differ from that of a healthy person. With adrenal insufficiency of a different etiology, the prognosis is determined by the underlying disease.

Mode

Addison's syndrome does not allow patients to engage in heavy physical labor. Any stressful condition (infection, physical or mental overstrain, etc.) requires an increase in the intake of glucocorticoids. The principle of dispensary observation in relation to these patients must be strictly observed. All patients with Addison's syndrome are supplied with a special memo, which indicates a rational schedule for taking corticosteroids and the optimal single and daily doses of various corticosteroid drugs for this patient. In case of an urgent situation, glucocorticoids for parenteral administration should be ready. Warning information should also be prepared for physicians in case the patient is unable to independently present information about his illness. Patients should be aware that they need to see a doctor immediately if they develop weakness, malaise, fever, abdominal pain, diarrhea, or other signs of deterioration. It is forbidden to drink alcohol, take barbituric sleeping pills, and use alkaline mineral waters to drink tablets containing corticosteroids.

1. Balabolkin M. I. Endocrinology, 1998 2. McDermott M. Secrets of Endocrinology, 2001 3. Wolfgang Oelkers M. Adrenal issuficiency. The New England Journal of Medicine, Vol. 335, no. 16, p. 1206-1212 http://www.temple.edu/imreports/Reading/Endo%20%20-Adrenal%20insuff.pdf 4. May M., Vaughn E., Carey R. Adrenocortical insufficiency - clinical aspects. In: Adrenal disorders, 1989, p. 171-189 5. Oelkers W., Diederich S., Bähr V. Recent advances in diagnosis and therapy of Addison’s disease. Journal of Endocrinology Vol. 1. 1994, p. 69-80