In Addison's disease, hypotension is the cause. Addison's disease: symptoms and features of the clinical picture

The disease is a rare pathology of the hormonal system. It is associated with the destruction of the structural adrenal cortex and dysfunction of the paired organ. Various infections, immune failure can provoke the development of the disease.

Pathology accompanies the adyssonian crisis - an acute condition in which poor adrenal function is manifested.

Depending on the causes and symptoms, Addison's disease is divided into forms:

• primary;
• secondary;
• iatrogenic.

The first form of Addison is characterized directly by insufficiency of the organ cortex. Symptoms are unclear or mild. For diagnosis, blood tests are performed.

In the secondary, there is a violation of the anterior pituitary gland. Insufficient production of adrenocorticotropic hormone develops. The production of the cortex of the paired organ decreases.

The iatrogenic form of Addison is characterized by atrophy of the paired organ. In the human body, the communication chains between the hypothalamus and the pituitary gland are disrupted. Addison's disease is diagnosed in young and middle-aged people.

Reasons for development

Most often, pathology develops with autoimmune damage to the tissues of the cortex of the organ.

Can lead to illness:

• tuberculosis;
• fungal infection;
• phospholipid deficiency;
• adrenoleukodystrophy;
• metastatic lesions.

Provokes the development of pathology disrupted work of cells of the immune system. Human lymphocytes begin to perceive tissues as foreign. An autoimmune process develops, lymphocytes secrete autoantibodies into the blood.

It's important to know! Tuberculosis, adrenolectomy and infections provoke the penetration of bacterial flora into the tissues of the adrenal glands. Addison's disease develops hematogenously. Once in the body, mycobacteria actively multiply and destroy structural cells.

Main manifestations and symptoms

The main manifestations of the disease include Addison's melasma - hyperpigmented spots. For this reason, Addison's disease is called bronze disease. In men, the symptoms of Addison's disease are manifested by severe irritability, psychosis, and disturbances in the activity of the cardiovascular system.

Also, the disease has the following symptoms:

• muscle weakness;
• darkening of the skin;
• decreased appetite;
• feeling unwell;
• paresthesia of the limbs;
• nausea and abdominal pain;
• orthostatic collapse;
• impaired sexual function;
• tachycardia.

The disease is manifested by increased irritability and tremor of the hands and feet. Very often, a sick person has a strong craving for salty and sour foods. The disease may be accompanied by high blood pressure and hypotension. Among the symptoms in women - a disturbed menstrual cycle, lack of menstruation.

Addisonian crisis

Addison's disease in terms of symptoms develops gradually. The acute manifestation of the disease is accompanied by the cessation of the production of corticosteroids. Or their synthesis can drop sharply in the body of a sick person.

The body begins to experience an acute deficiency in the necessary hormones. In an acute crisis, there is discomfort in the abdomen, pressure drops sharply, consciousness can be confused. Diarrhea, fainting, vomiting are possible. The patient should be sent to the clinic, Addison's crisis requires inpatient treatment.

Childbirth, pregnancy or incorrect drug therapy can provoke a pathological condition. Also, the factors that cause acute insufficiency include surgery, trauma, alcohol abuse. Infectious and inflammatory processes can cause complications.

The course of the disease in childhood

The disease is characterized in children by a sharp decrease in the body hormones aldosterone and cortisol. Birmer-Addison's disease manifests itself in children with the following symptoms of lethargy and sudden weight loss.

In an acute condition, the following manifestations are characteristic:

• anuria;
• vomit;
• diarrhea;
• abdominal pain;
• impossibility of movement;
• fall hell.

In children, the symptoms of Addison's disease can be severe. Hyperpigmentation, scleroderma, toxic goiter develop. If a child is not helped during a crisis, he may fall into a coma. In severe condition, seizures and other symptoms develop. Urgent care is required, it is important to stop the Addisonian crisis.

It is important to take into account all clinical recommendations during the Addisonian crisis and follow the diet during the illness.

Treatment

The therapeutic task in the treatment of the Adyssonic crisis is to support the body in the production of the necessary hormones. In an acute condition, hormonal substances are administered in an increased dosage intravenously and intramuscularly.

Drug treatment is prescribed with drugs from the groups:

• synthetic glucocorticoids;
• replacement mineralocorticoids.

To compensate for the deficiency of corticosteroids in the body of a sick person, the appointment of certain drugs that increase the level of hormones in the blood will help. Cortineff or Fludrocortisone are prescribed. The tablets are taken once a day in the morning. In some cases, the drug Konvuleks is prescribed for Addison's crisis.

The choice of the type of hormones depends on the course of the disease and the manifestation of symptoms. In some cases, hydrocortisone, prednisolone, dexamethasone are prescribed. If Addison's disease is caused by infection or tuberculosis, antibiotics and systemic antifungals are prescribed. With obvious signs of fluid retention in the body, the doctor prescribes decongestants.

Diet

Therapeutic nutrition is of great importance in the fight against pathology. Diet helps to stabilize hormonal levels and improve the synthesis of substances important for the body.

Note! With Addison's disease, there is a decrease in the acidity of the stomach. Therefore, it is necessary to consume easily digestible carbohydrates and an increased amount of salt. Chlorine ions contribute to an increase in acid in the gastric juice.

The delay in the amount of potassium in the body in pathology indicates that it is necessary to limit the diet of potatoes and foods with a high content of the substance. Dried apricots, beans, peas, raisins are excluded from the diet.

It is important to enrich the menu with vitamins and ascorbic acid. It is required to use rosehip decoctions, currants, yeast. You can not overload the stomach with heavy food. Patients often complain of nausea. The diet includes boiled and steam dishes.

Possible Complications

In Addison's disease, extremely severe conditions associated with insufficient hormone production can develop. The crisis can threaten the life of the patient. It is possible to reduce the level of glucose and sodium in the blood. This causes fainting, myalgia, general weakness and paresis of the limbs.

In some cases, manifestation of acute psychosis is possible. Pathology is characterized by increased excitability, irritability, confusion. With psychosis, it is required to calm the patient with medication.

Prevention

There is no specific prevention of the disease. In order not to encounter pathology, it is important to exclude stress and negative factors from life that lead to the development of pathology. It is required to lead a healthy lifestyle, exercise moderately, eat right and not abuse smoking and alcohol. Then Addison's bronze disease and its symptoms will not appear.

Anatomy
Adrenal gland (glandula suprarenalis) - a paired organ weighing about 12-13 g is located in the retroperitoneal space directly above the upper end of the corresponding kidney. The adrenal gland has the shape of a cone flattened from front to back, in which the anterior, posterior and renal surfaces are distinguished.
The adrenal glands are located at the level of 10-12 thoracic vertebrae. Right lies slightly lower than the left. With its posterior surface, the right adrenal gland is adjacent to the lumbar part of the diaphragm, the anterior one is in contact with the visceral surface of the liver and the duodenum, and the lower, concave renal surface is in contact with the upper end of the right kidney.
Left adrenal the anterior surface is adjacent to the tail of the pancreas and the cardial part of the stomach, its posterior surface is in contact with the diaphragm, and the lower surface is in contact with the upper end and medial edge of the left kidney. The right one is slightly smaller than the left one.

1 - diaphragm (diapragma); 2 - adrenal glands (gll. suprarenales); 3 - kidney (ren); 4 - abdominal aorta (aorta abdominalis); 5 - inferior vena cava (v. cava inferior); 6 - ureter (ureter)

Outside, the adrenal gland is covered with a fibrous capsule. The outer part of the parenchyma (represented by the outer layer of the cortical substance and the inner layer of the medulla, which makes up the inner part of the organ) of the organ is cortex, consisting of 3 zones: outside, closer to the capsule, is located zona glomerulosa, followed by the widest beam zone (zona fasciculata) and then the inner mesh zone (zona reticularis), on the border with the medulla.

The hormones of the adrenal cortex are called corticosteroids. They affect various types of metabolism, the immune system and the course of inflammatory processes. There are 3 groups of hormones:
♦ mineralocorticoids(aldosterone) produced by cells of the glomerular cortex;
♦ glucocorticoids(corticosterone, cortisol, hydrocortisol, cortisone), synthesized by the beam zone;
♦ sex hormones(androgen, estrogen, progesterone) produced by the cells of the reticular zone.

Located in the center of the adrenal gland brain matter (medulla), formed by large cells, among which there are cells that produce catecholamines: epinephrine and norepinephrine. They change the activity of the cardiovascular and nervous systems, the glandular epithelium, the processes of carbohydrate metabolism and thermogenesis.

Addison's disease(chronic adrenal insufficiency, or hypocorticism, bronze disease, English Addison's disease) is a rare endocrine disease, as a result of which the adrenal glands lose the ability to produce enough hormones, primarily cortisol. This pathological condition was first described by the British physician Thomas Addison in his 1855 publication entitled Constitutional and Local Consequences of Disease of the Adrenal Cortex.

Addison's disease occurs when more than 90% of the adrenal tissue is affected. In the vast majority of cases, the cause of the disease is an autoimmune process (an attack by one's own immune system), followed by tuberculosis in frequency. As a syndrome, chronic adrenal insufficiency is present in a variety of inherited diseases.

Insufficiency of the function of the adrenal cortex is acute and chronic. Chronic insufficiency of the adrenal cortex can be primary and secondary.
Primary insufficiency of the adrenal cortex occurs as a result of the destruction of the tissue of the adrenal gland itself, secondary insufficiency of the adrenal cortex is the result of a violation of the hypothalamic-pituitary regulation (production of adrenocorticotropic hormone of the pituitary gland and corticoliberin of the hypothalamus).
Secondary insufficiency of the adrenal cortex occurs in diseases of the brain with damage to the pituitary gland or hypothalamus (brain tumors, traumatic brain injury, after brain surgery, after radiation therapy, with various intoxications).

Causes of Addison's disease
* autoimmune damage to the adrenal cortex (attack by your own immune system)
*Tuberculosis of the adrenal glands.
* removal of the adrenal glands
* consequences of long-term hormone therapy
* fungal diseases (histoplasmosis, blastomycosis, coccidioidomycosis)
* sarcoidosis
* hemorrhages in the adrenal glands
* Tumors
* amyloidosis
* acquired immunodeficiency syndrome (AIDS)
* syphilis
* adrenoleukodystrophy.

Type of patient

clinical picture. Symptoms.
Addison's disease usually develops slowly, over several months or years, and its symptoms may go unnoticed or not appear until some stress or illness occurs that dramatically increases the body's need for glucocorticoids.

At the beginning:
There are complaints of weakness, fatigue, especially in the evening. Sometimes such weakness occurs only after physical exertion or stressful situations.
Appetite worsens
Patients often get colds
There is a poor tolerance of solar radiation, accompanied by persistent sunburn.

Stages of a detailed clinical picture:
Muscle weakness becomes more and more pronounced. It is difficult for the patient to carry out any movements. Even the voice becomes quiet. Chronic fatigue, gradually getting worse over time
Decreased body weight
Persistent hyperpigmentation (intensification of skin color) appears in the form of spots, especially in places of clothing friction, in open areas of the body exposed to sunburn ("Addison's melasma"), the color of the nipples, lips, cheeks intensifies.
There is a persistent decrease in blood pressure, which decreases even more in a standing position (orthostatic hypotension), and an increase in heart rate.
There are disorders of the gastrointestinal tract: nausea, vomiting, constipation, followed by diarrhea. Stomach ache
The amount of glucose in the blood decreases (hypoglycemia).
The work of the kidneys is disturbed, often manifested by nighttime urination.
On the part of the central nervous system, there are disturbances in attention, memory, depressive states, irritability, irascibility, dissatisfaction with everything, and depression.
Craving for salt and salty foods, thirst, drinking plenty of fluids
In women, due to a lack of androgens, pubic and axillary hair falls out, violation or disappearance of the menstrual cycle, in men - impotence.
Tetany (convulsive attacks caused by impaired calcium metabolism in the body) (especially after drinking milk) due to excess phosphate
Paresthesia (sensory disturbance characterized by sensations of numbness, tingling, crawling, and sensory disturbances in the extremities), sometimes to the point of paralysis, due to excess potassium
Excessive amount of urine (oliguria)
Hypovolemia (decreased blood volume)
Dehydration (dehydration of the body)
Tremor (shaking hands, head)
Dysphagia (swallowing disorders)

Manifestation of Addison's disease in the oral cavity

Addisonian crisis
In some cases, the symptoms of Addison's disease can come on unexpectedly quickly. This state of acute insufficiency of the adrenal cortex is called "addisonian crisis" and is an extremely dangerous, life-threatening condition for the patient. Any acute illness, blood loss, trauma, surgery, or infection can exacerbate an existing adrenal insufficiency, which can lead to an Addisonian crisis. Addisonian crises are most common in undiagnosed or untreated, or inappropriately low, underdosed corticosteroid patients with Addison's disease, or in those in whom the dose of glucocorticoids has not been temporarily increased due to illness, stress, surgery, etc.

In previously diagnosed and adequately treated patients, Addisonian crisis may occur as a result of an abrupt cessation of corticosteroid treatment or a sharp decrease in their dose, or an increase in the body's need for glucocorticoids (surgery, infections, stress, trauma, shock).

Also, the causes of the crisis are bilateral hemorrhage in the adrenal glands, bilateral embolism of the adrenal arteries or thrombosis of the adrenal veins (for example, during X-ray contrast studies), removal of the adrenal glands without adequate replacement therapy.

An Addisonian crisis can also occur in patients who do not suffer from Addison's disease, but who receive or have received in the recent past long-term treatment with glucocorticoids for other diseases (inflammatory, allergic, autoimmune, etc.) with a sharp decrease in the dose or abrupt withdrawal of glucocorticoids, as well as with an increase in the body's need for glucocorticoids. The reason for this is the inhibition of the secretion of ACTH and endogenous glucocorticoids by exogenous glucocorticoids, the gradually developing functional atrophy of the adrenal cortex with prolonged glucocorticoid treatment, as well as a decrease in the sensitivity of tissue receptors to glucocorticoids (desensitization) during therapy with supraphysiological doses, which leads to the patient's dependence on the intake of exogenous glucocorticoids in the body. (“steroid addiction”).

Addisonian crisis symptoms
* Sudden severe pain in the legs, lower back or abdomen;
* Severe vomiting, diarrhea, leading to dehydration and the development of shock;
* a sharp decrease in blood pressure;
* loss of consciousness;
* Acute psychosis or confusion, delirium (a mental disorder that occurs with impaired consciousness (from a clouded state to a coma));
* a sharp decrease in blood glucose levels;
* Hyponatremia, hyperkalemia, hypercalcemia, hyperphosphatemia;
* Brown plaque on the tongue and teeth due to hemolysis and the development of iron deficiency.

Treatment.
Diet for Addison's disease: a sufficient amount of proteins, fats, carbohydrates and vitamins, especially C and B (recommend rosehip broth, black currant, brewer's yeast). Table salt is consumed in an increased amount (20 g / day). The diet reduces the content of potatoes, peas, beans, beans, dried fruits, coffee, cocoa, chocolate, nuts, mushrooms. Vegetables, meat, fish must be consumed boiled. The diet is fractional, a light dinner (a glass of milk) is recommended before bedtime.
The treatment for Addison's disease is adrenal hormone replacement therapy. Apply hydrocortisone (with a lack of cortisol) and fludrocortisone (with a lack of aldosterone) - when using this hormone, it is advised to increase the amount of salt. Hydrocortisone 10 mg in the morning and 5 mg orally daily in the afternoon (adults up to 20-30 mg / day). Fludrocortisone 0.1-0.2 mg orally once a day. With an increase in blood pressure, its dose should be reduced. In acute illness (for example, a cold) or after a minor injury, the dose of hormones is doubled until you feel better. During surgical treatment before and (if necessary) after surgery, the dose of hormones is corrected. In liver diseases, as well as in elderly patients, the doses of drugs should be reduced.
During an Addisonian crisis, there is a drop in blood pressure and blood glucose, as well as an increase in potassium, which can threaten the patient's life. Treatment of an adrenal crisis: urgent hospitalization is shown. 0.9% sodium chloride solution is administered intravenously until dehydration is eliminated, hydrocortisone and dextrose (sugar). Clinical improvement (as measured primarily by blood pressure recovery) usually occurs 4 to 6 hours after intravenous therapy. When the patient is able to drink and take drugs by mouth, the amount of hydrocortisone is reduced, keeping only the maintenance dose. With a lack of aldosterone, maintenance therapy with fludrocortisone acetate is prescribed.
When the temperature rises (against the background of normal blood pressure), antipyretics, such as paracetamol, are prescribed
In surgical interventions, it is necessary to adjust the dose of steroid hormones
Infectious diseases should be avoided.

Diagnostics
It is carried out on the basis of complaints and the appearance of patients, a persistent decrease in blood pressure, poor exercise tolerance. In the blood of patients found:
* the adequacy of steroid therapy is checked (if any)
* BP↓
* Urea and electrolytes: K (potassium), Na (sodium) ↓
* sugar↓
*serum cortisol levels ↓
* there is no reaction to the test with synacthen
* ACTH (adrenocorticotropic hormone) levels are high
* determination of antibodies to the adrenal glands

Heredity
There are several different causes that can lead to the development of Addison's disease, and some of them have a hereditary component. The most common cause of Addison's disease in the United States and Western Europe is autoimmune destruction of the adrenal cortex. The tendency to develop this autoimmune aggression against the tissues of one's own adrenal glands is most likely inherited as a complex genetic defect. This means that for the development of such a condition, an “orchestra” of several different genes is needed, interacting with as yet unidentified environmental factors.

Forecast
With adequate treatment of Addison's disease, the prognosis is favorable. Life expectancy is close to normal.

Addison's disease and dermatophytosis as a complication of permanent corticosteroid therapy
The patient suffered from Addison's disease for about 20 years, the disease began with inexplicable weakness and the appearance of pigmentation of the palms with a bronzed tint.
She has been monitored by an endocrinologist, has been receiving maintenance corticosteroid therapy for many years, and in recent years - metipred one tablet a day.
About a year ago I went to a dermatologist for a rash on my face. A fungal infection was suspected, scrapings were taken for pathogenic fungi. However, no pathogenic fungi were found in the obtained scrapings, and the doctor rejected this assumption ...
The doctor prescribed a corticosteroid ointment, the use of which causes an exacerbation of the skin process. Some relief is provided by Triderm ointment.
At the reception I was asked to help in the diagnosis and treatment. In addition, she has a large family, children and grandchildren, the patient is afraid that she is contagious to others?
On examination: even "from the threshold" the diagnosis was already clear - dermatophytosis of the smooth skin of the face.
Rashes are represented by papular elements and spots located on the left cheek in the form of an annular plaque with scalloped borders.
The patient shows the area of ​​the lesion on herself, while clearly demonstrating the fungal infection of the nail plates of the hands.
Further examination revealed foci of mild erythema and papular rash on the skin of the right breast. The rash is barely defined, but the scalloped borders of the rash are still clearly visible.
Totally all the nail plates of the feet are affected.
The entire skin of the patient is thinned, atrophic, numerous telangiectasias on the face. Cannon hypertrichosis is noted. Integuments of the increased swarthyness.
In addition, the patient notes that on days when metipred is missed, a sharp weakness develops.

Clinical diagnosis
Addison's disease and dermatophytosis of the skin and nails as complications of permanent corticosteroid therapy.

Addison's disease(also known as chronic, or hypocorticism) is rare. This affects the production of two hormones - cortisol and aldosterone - that help regulate blood pressure in the body.

adrenal glands

The adrenal glands are two small triangular glands located on top of the kidneys, high in the back of the abdominal wall (see photo).

Each gland has an inner and outer layer, which have separate functions:

• the inner region (medulla) produces the hormone adrenaline, and
• the outer layer (cortex) produces steroid hormones and small amounts of male and female sex hormones, testosterone and estrogen.

In Addison's disease, as a rule, the cortex of both adrenal glands is destroyed. This disrupts the production of steroid hormones, cortisol and aldosterone.

cortisol

Autoimmune diseases

The immune system protects the body from infections and diseases. If a person becomes ill, the immune system will begin to produce antibodies (a special type of protein to neutralize or destroy disease-causing organisms and toxins). These antibodies will attack the cause of the illness.

Addison's disease is a disorder in which the adrenal glands do not produce enough hormones. Along with a significant decrease in cortisol levels, there is often a decrease in aldosterone production.

The disorder is also called "adrenal insufficiency". It is diagnosed in patients of all age groups and both sexes. In some cases, the disease can be life threatening.

Treatment consists of taking hormones produced by the adrenal glands in reduced amounts. Hormone therapy allows you to achieve the same beneficial effect that is observed with the normal (natural) production of the missing biologically active substances.

Symptoms

If a patient is diagnosed with Addison's disease, the symptoms most likely first appeared several months before the visit to the doctor. Signs of pathology appear extremely slowly and may include the following conditions:

• fatigue and muscle weakness;
• unplanned weight loss and decreased appetite;
• darkening of the skin (hyperpigmentation);
• low blood pressure, prone to fainting;
• an acute desire to eat salty foods;
• low blood sugar (hypoglycemia);
• nausea, vomiting, diarrhea;
• pain in muscles or ligaments;
• irritability, aggressiveness;
• depressive state;
• body hair loss or sexual dysfunction (in women).

Acute adrenal insufficiency (adrenal crisis)

However, in some cases, the pathology occurs suddenly. Adrenal crisis (acute adrenal insufficiency) is Addison's disease, the symptoms of which may be as follows:

• pain in the lower back, abdomen, or legs;
• severe vomiting or diarrhea leading to dehydration;
• low blood pressure;
• elevated potassium levels (hyperkalemia).

When to See a Doctor

It is necessary to visit a specialist and conduct a diagnosis if you notice signs and symptoms characteristic of Addison's disease. Combinations of signs from the list below are especially dangerous:

• darkening of areas of the skin (hyperpigmentation);
• a strong feeling of fatigue;
• unintentional weight loss;
• problems with the functioning of the gastrointestinal tract (including abdominal pain);
• dizziness or fainting;
• cravings for salty foods;
• muscle or joint pain.

Causes

Addison's disease occurs as a result of a malfunction of the adrenal glands. Most often this happens when the adrenal glands are damaged, because of which the latter produce an insufficient amount of cortisol, as well as aldosterone. These glands are located just above the kidneys and are part of the endocrine system. The hormones they produce regulate the functioning of almost all organs and tissues of the body.

The adrenal glands are made up of two sections. Their inner part (medulla) synthesizes dopamine and norepinephrine. The outer layer (cortex) produces a group of hormones called corticosteroids. This group includes glucocorticoids, mineralocorticoids and male sex hormones - androgens. Substances of the first two types are necessary to maintain the vital activity of the body.

• Glucocorticoids, including cortisol, interfere with the body's ability to convert food nutrients into energy. They also play an important role in preventing inflammation and generating normal responses to stressors.
• Mineralocorticoids, including aldosterone, maintain a natural balance of sodium and potassium, which maintains normal blood pressure.
• Androgens are produced in small amounts in both men and women. They are responsible for the sexual development of men and affect the growth of muscle tissue. In both sexes, these hormones regulate libido (sex drive) and create a sense of satisfaction with life.

Primary adrenal insufficiency

Addison's disease occurs when the adrenal cortex is damaged and the level of hormones produced decreases. In this case, the pathology is called primary adrenal insufficiency.

Most often, the impossibility of producing glucocorticoids and mineralocorticoids in a normal volume is caused by an erroneous attack of the body on itself, that is, an autoimmune disease. For unknown reasons, the immune system begins to view the adrenal cortex as a foreign element that must be destroyed.

In addition, cortical damage can be triggered by the following factors:

• tuberculosis;
• infections of the adrenal glands;
• spread of cancer (metastases) to the adrenal glands;
• hemorrhage.

Secondary adrenal insufficiency

Sometimes the pathology occurs without damage to the adrenal glands, and doctors diagnose "Addison's disease", the causes of which are hidden in the dysfunction of the pituitary gland. This brain appendage produces adrenocorticotropic hormone (ACTH), which stimulates the production of these substances by the adrenal cortex. Violation of the production of ACTH leads to a decrease in the level of hormones produced by the adrenal glands, even if they are not damaged. This condition is called secondary adrenocortical (adrenal) insufficiency.

A similar pathology can also occur due to the abrupt cessation of hormone therapy, in particular, if the patient took corticosteroids to treat chronic diseases such as asthma or arthritis.

adrenal crisis

If you have Addison's disease and neglect treatment, an adrenal crisis can be triggered by physical stress, such as an injury, infection, or illness.

Before visiting a doctor

Before visiting a doctor with a narrow specialization, it is necessary to consult a general practitioner. It is he who, if necessary, will redirect you to an endocrinologist.

Since medical consultations are often extremely brief, it is best to prepare for your doctor's visit in advance. Recommended:

• Make inquiries ahead of time and find out if there are any dietary or lifestyle restrictions that need to be observed before visiting a specialist.
• In writing, record any symptoms noticed, including signs of illness and abnormalities that at first glance are not related to the topic of the appointment.
• Write down key personal information, including major stressors or recent lifestyle changes.
• Make a list of all medicines, dietary supplements and fortified preparations that you are currently taking.
• Take a relative or friend with you (if possible). It is not uncommon for patients to forget essential details noted by a doctor during a medical consultation. The escort will be able to record the most important information and will not let you forget the names of the recommended medicines.
• Make a list of questions you plan to ask the specialist.

The duration of medical consultation is limited, and Addison's disease is a serious disorder that requires prompt treatment. Make a list of your questions in such a way that first of all draw the attention of the specialist to the most important points. You might want to know the following:

• What exactly is the root cause of the symptoms or uncharacteristic condition?
• If this isn't Addison's disease, what is it? Are there alternative reasons for the deterioration of well-being?
• What examinations will need to be done?
• Is my condition temporary? Or has it become a chronic disease?
• What is the best thing to do in my situation?
• Are there alternative methods of treatment, or do you categorically insist on the method you proposed?
• I have other chronic diseases. If I have Addison's disease, how can I treat all pathologies at the same time?
• Do I need to see a more specialized doctor?
• Is it possible to purchase a cheaper analogue of the drug recommended by you?
• Can I bring themed brochures or other printed materials with me? What sites on the Internet do you recommend?
• Are there any restrictions that must be observed? Can you drink alcohol with Addison's disease?

Feel free to ask any other questions, including any that came to mind during your consultation at the clinic.

What will the doctor say

The doctor will ask you a series of questions of his own. It is better to prepare for them in advance in order to devote enough time to the most important problems. The specialist is likely to be interested in the following details:

• When did you first notice symptoms of the disease?
• How are the symptoms of pathology manifested? Do they occur spontaneously or do they last for some period of time?
• How intense are the symptoms?
• What do you think improves your condition?
• Are there any factors contributing to the deterioration of your condition?

Diagnostics

First, the doctor will review your medical history and current signs and symptoms. If adrenocortical insufficiency is suspected, he may prescribe the following examinations:

• Blood test. Measuring the levels of sodium, potassium, cortisol and adrenocorticotropic hormone in the blood will allow the specialist to obtain primary data on possible adrenal insufficiency. In addition, if Addison's disease is caused by an autoimmune disorder, corresponding antibodies will be found in the blood.
• stimulation of ACTH. The purpose of this study is to measure cortisol levels before and after synthetic adrenocorticotropic hormone injection. The latter signals the adrenal glands to produce additional cortisol. If the glands are damaged, insufficient volume of the substance will be obvious in the results of the study - it is possible that the adrenal glands will not respond at all to the injection of ACTH.
• Test for hypoglycemia with insulin administration. This study is effective in cases where the doctor reasonably suspects that the patient has secondary adrenocortical insufficiency caused by pituitary disease. The analysis includes several tests of blood sugar (glucose) and cortisol levels. Each check is carried out after a certain time period after the introduction of a dose of insulin. In healthy people, glucose levels decrease and cortisol levels rise accordingly.
• Imaging studies. Your doctor may order a computed tomography (CT) scan to take a picture of your abdomen. This picture checks the size of the adrenal glands and confirms the presence or absence of anomalies that can lead to the development of a disorder such as Addison's disease. Diagnosis is also made by MRI if secondary adrenocortical insufficiency is suspected. In this case, a picture of the pituitary gland is taken.

Treatment

Regardless of the cause and symptoms, the treatment of adrenocortical insufficiency is carried out in the form of hormonal therapy in order to correct the level of missing steroid hormones. There are several treatments, but the most common are:

• Taking corticosteroids by mouth (by mouth). Some doctors prescribe fludrocortisone to replace aldosterone. Hydrocortisone (Cortef), prednisone, or cortisone acetate is used to replace cortisol.
• Corticosteroid injections. If the patient suffers from severe vomiting and is unable to take medications by mouth, hormone injections will be required.
• Replacement therapy for androgen deficiency. Dehydroepiandrosterone is used to treat androgen deficiency in women. Although Addison's disease (photos showing the symptoms of the pathology are presented on the page) most often consists in lowering the level of cortisol, and not androgens, a violation of the production of the latter can lead to a further deterioration in the patient's condition. In addition, according to some studies, replacement therapy with a lack of male sex hormones in women improves overall well-being, brings a sense of satisfaction with life, increases libido and sexual satisfaction.

Higher doses of sodium are also recommended, especially during strenuous exercise, hot weather, or stomach or intestinal disorders (eg, diarrhea). The doctor will also suggest increasing the dosage if the patient is in a stressful situation - for example, has undergone surgery, an infection, or a relatively sluggish illness.

Treatment of an adrenal crisis

An adrenal (adrenocortical) crisis is a life-threatening condition. Addison's disease, which is treated too late, can lead to a sharp drop in blood pressure, low sugar levels and increased levels of potassium in the blood. In this case, you must immediately seek medical help. Treatment most often consists of intravenous hydrocortisone, saline, and

Addison's disease is a fairly rare endocrine pathology. Its development is based on destruction ( destruction) tissues of the outer ( cortical) adrenal layer. Such destruction can be provoked by various factors. They can be harmful bacteria e.g. mycobacteria), fungi ( candida, cryptococcus), viruses ( cytomegalovirus, herpes, etc.), genetic, immune, microcirculatory ( violation of the blood supply to the adrenal cortex) violations ( ).

Damage to the adrenal cortex leads to impaired secretion of steroid hormones, primarily aldosterone and cortisol, which regulate water-salt, protein, carbohydrate, and lipid metabolism in the body. They are also involved in the implementation of stress reactions, affect blood pressure, the total volume of circulating blood.

With Addison's disease, various symptoms may occur, such as increased fatigue, general weakness, dizziness, decreased ability to work, headache, thirst, cravings for salty foods, muscle weakness, myalgia ( muscle pain), muscle cramps, abdominal pain, nausea, vomiting, menstrual irregularities, low blood pressure, hyperpigmentation ( skin darkening), vitiligo, tachycardia ( cardiopalmus), pain in the heart, etc.

The structure and functions of the adrenal glands

The adrenal glands are paired endocrine glands. Each adrenal gland right or left) is placed on the superomedial ( top inner side) surface of the upper end ( poles) of the corresponding kidney ( right or left). Both adrenal glands have approximately the same mass ( about 7 - 20 g each). In children, the adrenal glands weigh slightly less ( 6 g). These glands are located in the retroperitoneal fatty tissue at the level of the XI-XII thoracic vertebrae. Retroperitoneal tissue is located in the retroperitoneal space - an area located behind the posterior leaf of the parietal ( parietal) peritoneum lining the posterior wall of the abdominal cavity. The retroperitoneal space extends from the diaphragm ( respiratory muscle that separates the thoracic and abdominal cavities) to the small pelvis ( anatomical region just below the abdominal cavity). In addition to the adrenal glands, it contains the kidneys, pancreas, aorta, inferior vena cava and other organs.

The left adrenal gland has a semilunar shape, the right one is triangular. In each of them, the anterior, posterior and renal surfaces are distinguished. With their back surface, both adrenal glands are adjacent to the diaphragm. Renal ( lower) their surface is in contact with the upper pole of the corresponding kidney. The right adrenal gland in the retroperitoneal space is located slightly higher than the left. With its front surface, it adjoins the inferior vena cava, liver and peritoneum. The left adrenal gland contacts the pancreas, the cardia of the stomach, and the spleen anteriorly. On the anteromedial ( front inner side) the surface of each of the adrenal glands is the so-called gate ( hilum), through which of them ( from the adrenal glands) exit the central veins ( hereinafter referred to as the adrenal veins).

Each adrenal gland has one central vein. The left adrenal vein then drains into the left renal vein. Venous blood from the right adrenal gland is transported through the right adrenal vein immediately into the inferior vena cava. In the gates of the adrenal glands, lymphatic vessels can also be found, through which the lymphatic fluid enters the lumbar lymph nodes ( nodi lymphatici lumbales) located around the abdominal aorta and inferior vena cava.

Arterial blood enters the adrenal glands through branches of the superior, middle, and inferior adrenal arteries ( a. suprarenalis superior, a. suprarenalis media, a. suprarenalis inferior). The superior adrenal artery is a continuation of the inferior phrenic artery. The middle adrenal artery arises from the abdominal aorta. The inferior adrenal artery serves as a branch of the renal artery. All three adrenal arteries under the connective tissue capsule that covers each of the adrenal glands form a dense arterial network. Small vessels depart from this network ( about 20 - 30), penetrating into the thickness of the adrenal glands through their anterior and posterior surfaces. Thus, arterial blood enters the adrenal glands through numerous vessels, while venous blood is removed from these endocrine glands through only one vessel - the central ( adrenal) vein.

The innervation of the adrenal glands is carried out due to the branches extending from the solar, renal and adrenal nerve plexuses, as well as the branches of the phrenic and vagus nerves.

Inward from the connective tissue capsule is the cortical substance ( outer layer) adrenal gland, which accounts for about 90% of the entire parenchyma ( fabrics) of this body. The remaining 10% in the adrenal gland is occupied by its medulla ( inner layer of the adrenal gland), which is located under the cortical substance, directly, in the very depths of the gland. The cortex and medulla have a different structure, functions and embryonic origin. cortex ( adrenal cortex) is represented by loose connective and glandular tissue. This layer on anatomical sections has a yellowish-brown color.

The outer layer of each of the adrenal glands is usually divided into three zones - glomerular, fascicular and reticular. The glomerular zone is the outermost layer of the cortical substance and is localized directly under the adrenal capsule. The reticular zone borders on the adrenal medulla. The bundle zone occupies a middle position between the glomerular and reticular. The zona glomeruli produce mineralocorticoids ( ), in the bundle - glucocorticosteroids ( cortisol and cortisone), and in the reticular - androgens. The adrenal medulla has a brownish-red color and is not divided into any zones. In this area, catecholamines are synthesized in the adrenal glands ( epinephrine and norepinephrine).

The adrenal glands are vital organs and perform a variety of functions through the production of a number of hormones with specific properties. As mentioned above, mineralocorticoids are produced in these endocrine glands ( aldosterone, deoxycorticosterone, corticosterone), glucocorticosteroids ( cortisol and cortisone), androgens and catecholamines ( epinephrine and norepinephrine). The secretion of aldosterone and cortisol by the adrenal glands is vital for a person. Aldosterone is the only mineralocorticoid that the adrenal glands secrete into the blood. This steroid hormone contributes to the retention of sodium, chlorides and water in the body and the excretion of potassium along with urine. It contributes to an increase in systemic arterial pressure, the total volume of circulating blood, affects its acid-base state and osmolarity. Aldosterone regulates the proper functioning of the sweat and gastrointestinal glands.

Cortisol, like aldosterone, is also a steroid hormone. It has a wide range of effects on metabolism metabolism) in organs and tissues of the body. Cortisol stimulates the formation of large amounts of glucose and glycogen in the liver and inhibits ( blocks) their utilization in peripheral tissues. This contributes to the development of hyperglycemia ( an increase in blood glucose levels). In adipose, lymphoid, bone and muscle tissue, this hormone stimulates the breakdown of proteins. In the liver, on the contrary, it activates the synthesis of new proteins. Cortisol also regulates fat metabolism. In particular, it promotes the breakdown of fats in some tissues ( for example, fatty) and lipogenesis ( formation of new fats) in others ( torso, face). This glucocorticoid is the main stress hormone that helps the body adapt to the action of various stress factors ( infections, physical overexertion, mental or mechanical injuries, surgical interventions, etc.).

Androgens are produced by the cells of the reticular cortex of the adrenal glands. Their main representatives are dehydroepiandrosterone, etiocholanolone, androstenediol and androstenedione.

Testosterone and estrogens are practically not produced by the adrenal glands. Androgens, which are produced in large quantities in the adrenal glands, have a much smaller effect on body tissues than do the main sex hormones. So, for example, testosterone in its activity exceeds the action of androstenedione by 10 times. Androgens are responsible for the development of secondary sexual characteristics, such as voice change, body hair, development of genital organs, etc., regulate metabolism, increase libido, that is, sexual desire.

Catecholamines ( epinephrine and norepinephrine), which are formed in the adrenal medulla, are responsible for the adaptation of the body to acute environmental stressors. These hormones increase heart rate and regulate blood pressure. They are also involved in tissue metabolism ( metabolism), through inhibition of insulin release ( a hormone that regulates blood glucose levels) from the pancreas, activation of lipolysis ( fat breakdown) in adipose tissue and breakdown of glycogen in the liver.

Causes of Addison's Disease

Addison's disease is an endocrine disease that occurs as a result of the destruction of the tissues of the adrenal cortex under the influence of certain harmful factors. In another way, this disease is also called primary hypocorticism ( or primary adrenal insufficiency). This pathology is a fairly rare disease and, according to statistics, occurs only in 50 - 100 new cases per 1 million adults per year. Primary hypocorticism occurs much more often than secondary.

Secondary adrenal insufficiency is a separate endocrine disease and does not belong to Addison's disease, as it appears as a result of a violation of the secretion of adrenocorticotropic hormone by the pituitary gland ( ACTH), which serves as a natural stimulant for the functioning of the adrenal cortex. This hormone controls the production and secretion of hormones ( mainly glucocorticosteroids and androgens) adrenal cortex. In conditions of ACTH deficiency, bundle ( average) and mesh ( internal) zones of the adrenal cortex undergo gradual atrophy, which leads to adrenal insufficiency, but already secondary ( since the root cause of the disease is not in the adrenal glands themselves).

In Addison's disease, all three areas of the cortex are affected at once ( cortex) adrenal glands - glomerular, fascicular and reticular, therefore, it is believed that primary hypocorticism is clinically more severe than secondary. It should also be noted that all the symptoms that occur in a patient with Addison's disease are associated only with the destruction of the adrenal cortex, and not their medulla, the possible destruction of which ( depending on the cause of primary hypocorticism) does not play any role in the mechanism of development of this pathology. As mentioned a little above, Addison's disease occurs as a result of the action of certain damaging factors on the adrenal cortex. They can be various microorganisms ( bacteria, fungi, viruses), autoimmune processes, neoplasms ( tumor or metastatic lesion of the adrenal glands), genetic disorders ( e.g. adrenoleukodystrophy), impaired blood supply to the adrenal cortex ( DIC, antiphospholipid syndrome).

The most common causes of Addison's disease are:

• autoimmune damage to the tissues of the adrenal cortex;
• adrenalectomy;
• metastatic lesion of the adrenal cortex;
• fungal infections;
• adrenoleukodystrophy;
• antiphospholipid syndrome;
• DIC;
• Waterhouse-Frideriksen syndrome.

Autoimmune damage to the tissues of the adrenal cortex

In the vast majority of all new cases ( in 80 - 90%) Addison's disease occurs as a result of autoimmune destruction of the adrenal cortex. Such destruction appears due to a violation of the normal development of the immune system. cells of the immune system lymphocytes) in such patients upon contact with adrenal tissue ( through the blood) begin to perceive it as alien. Because of this, they are activated and initiate the destruction of the adrenal cortex. An important role in the process of autoimmune destruction is played by autoantibodies secreted into the blood by lymphocytes.

Autoantibodies are antibodies ( protein, protective molecules) directed against their own tissues ( in this case against the tissues of the adrenal cortex), which specifically bind to various structures on the cells of the adrenal cortex and, thereby, cause their death. The main types of autoantibodies observed in the blood of patients with autoimmune Addison's disease are antibodies to adrenal steroidogenesis enzymes ( )-21-hydroxylase ( P450c21), 17a-hydroxylase ( P450c17P450scc).

Tuberculosis

Tuberculosis is an infectious disease caused by mycobacteria ( usually Mycobacterium tuberculosis). Tuberculosis of the adrenal glands is the second most common cause of primary hypocorticism. In the vast majority of cases, this form of tuberculosis is secondary, that is, infection of the tissues of the adrenal cortex occurs already in the presence of a tuberculosis focus in the patient's body, which is located in some other organ ( e.g. lungs, bones, liver, kidneys, etc.). Infection transfer ( harmful mycobacteria) to the adrenal glands from the primary affected organs most often occurs hematogenously ( by blood). Getting inside the adrenal glands, mycobacteria begin to multiply and destroy their normal tissue, while destruction is often noted not only of the cortex, but also of the medulla of these endocrine glands. The first symptoms of Addison's disease, provoked by tuberculosis, begin to occur in a patient only after mycobacteria destroy a significant proportion of the tissues of the adrenal cortex ( about 80 - 90%). That is why it is quite difficult to diagnose this form of the disease in the early stages.

Adrenalectomy

Addison's disease can also occur as a result of bilateral adrenalectomy. Adrenalectomy is the surgical removal of one or both adrenal glands for medical reasons. Most often, bilateral adrenalectomy is performed in patients with a tumor lesion of both adrenal glands. Removal of two adrenal glands is often carried out in persons suffering from Itsenko-Cushing's disease. In this endocrine disease, pituitary tissues produce adrenocorticotropic hormone in excess ( ACTH), which makes the adrenal cortex work more intensively, which is accompanied by the development of hypercortisolism ( increased secretion of hormones by the adrenal cortex).

Metastatic lesion of the adrenal cortex

In some cases, the adrenal cortex can be affected by tumor metastases. Metastasis is a situation in which tumor cells from a primary cancer site located in one organ are brought into the blood stream ( or, for example, through the lymph) to other organs ( not necessarily adjacent). Having penetrated into secondary organs, malignant cells begin to multiply intensively, which is accompanied by the formation of new ones ( but already secondary - child) tumors, which are called metastases. Metastasis is one of the complications of most malignant neoplasms and is absolutely not typical for benign tumors. Metastases in the adrenal cortex are observed in 57% of cases of breast cancer, in 32% of cases of melanoma ( malignant tumor of pigment cells - melanocytes), in 38% of cases of bronchogenic lung cancer. Also a fairly common cause of Addison's disease associated with metastatic lesions of the adrenal cortex is non-Hodgkin's large cell lymphoma ( malignant disease of the blood).

Fungal infections

A fungal infection is rarely the cause of Addison's disease. Primary hypocorticism is most often observed with paracoccidioidomycosis, coccidioidomycosis, systemic ( widespread) histoplasmosis, candidiasis, blastomycosis, cryptococcosis. The most common cause of Addison's disease of all the above types of mycoses is paracoccidioidomycosis, caused by fungi of the genus Paracoccidioides Brasiliensis. This type of mycosis is mainly found in the population of Latin America. With paracoccidioidomycosis, the lungs, mucous membrane of the upper respiratory tract, lymph nodes, and in some cases the skin and adrenal glands are affected. In tissues damaged by harmful fungi, granulomas are formed ( inflammatory nodules), microabscesses ( small cavities filled with pus) and areas of focal necrosis ( dead tissue areas).

HIV infection

The patient has HIV infection ( disease resulting from infection with the human immunodeficiency virus) may be the cause of Addison's disease. The fact is that with HIV infection, patients develop a serious immunodeficiency state, in which their body fights hard enough against any infections, as a result of which they often suffer from a variety of infectious diseases. The presence of impaired immunity and frequent systemic infections ( e.g. fungal infections, cytomegalovirus infection, etc.) under certain circumstances can cause primary hypocorticism ( since the presence in the body of any infection, sooner or later, will lead to damage to the tissues of the adrenal cortex). It is believed that in patients with HIV infection latently ( is hidden) ongoing Addison's disease is noted in 8 - 11% of all cases.

Adrenoleukodystrophy

Adrenoleukodystrophy ( ALD) is an inherited disease resulting from a deletion ( removal) a specific region on the long arm of the X chromosome. The ALD gene is localized in this region, which encodes the structure of the enzyme lignoceroyl-CoA synthetase, which is involved in the oxidation of long-chain fatty acids ( JK) in the cells of the central nervous system, adrenal glands and other tissues. With adrenoleukodystrophy, this enzyme is not produced, as a result of which a large amount of fat accumulates in the nervous tissue and in the adrenal cortex ( JFA along with cholesterol esters), which gradually leads to their degeneration and death. This pathology is most often registered in men ( adrenoleukodystrophy is an X-linked recessive disease).

There are various clinical variants of the course of adrenoleukodystrophy, and not always with this disease, the tissues of the adrenal cortex can be seriously affected. For example, in some of these patients, neurological symptoms predominate ( juvenile form of adrenoleukodystrophy), while signs of Addison's disease may be absent for a long time ( especially at the beginning of the disease).

In addition to the adrenal glands and the central nervous system, adrenoleukodystrophy often develops primary male hypogonadism ( testicular failure), which can affect the reproductive capacity of men. Adrenoleukodystrophy is the third leading cause of Addison's disease ( after autoimmune destruction of the adrenal cortex and adrenal tuberculosis). The associated neurological symptoms associated with ALD make the prognosis of Addison's disease less favorable than other causes of primary hypocorticism.

Antiphospholipid Syndrome

antiphospholipid syndrome ( APS) is a pathological condition characterized by a violation of blood clotting and the appearance of blood clots in various vessels. APS is caused by antibodies ( protein, protective molecules) to phospholipids of cell membranes of platelets, endotheliocytes ( cells that line the inner surface of blood vessels). It is believed that the occurrence of such antibodies in the blood of patients is associated with a malfunction of the immune system.

Antiphospholipid antibodies have a direct damaging effect on the endothelium ( inner wall) vessels, inhibit the production of special proteins by endotheliocytes ( prostacyclin, thrombomodulin, antithrombin III) possessing anticoagulant ( anticoagulants) properties, as a result of which they ( antibodies) contribute to the development of thrombosis. In the antiphospholipid syndrome, in very rare cases, bilateral adrenal vein thrombosis can occur, which can cause damage to the tissues of the adrenal cortex ( due to impaired venous outflow from the tissues of the adrenal glands) and the development of Addison's disease.

DIC

The tissues of the adrenal cortex are quite often affected in the syndrome of disseminated intravascular coagulation ( DIC), which is one of the pathologies of the hemostasis system ( system that controls the liquid state of the blood and stops bleeding). At the first stages of DIC, numerous small blood clots form in various tissues and organs ( blood hypercoagulability), which subsequently ( in the later stages of this syndrome) is complicated by consumption coagulopathy ( i.e. exhaustion of the coagulation system), accompanied by a severe violation of blood coagulation and the development of hemorrhagic syndrome ( Spontaneous difficult-to-stop bleeding occurs in various organs and tissues).

The causes of DIC can be a variety of conditions, such as severe mechanical trauma, hemolytic anemia ( decrease in the number of red blood cells in the background of their excessive destruction), leukemia, tumors, systemic bacterial, viral, fungal infections, autoimmune diseases, intrauterine fetal death, premature placental abruption, transfusion of incompatible blood, etc. With DIC, numerous small blood clots can be found in the vessels of the tissues of the adrenal cortex, which serve as a direct cause microcirculation disorders, circulatory disorders, cell death of the glomerular, fascicular and reticular zones and the development of primary adrenal insufficiency ( Addison's disease).

Waterhouse-Frideriksen Syndrome

Waterhouse-Friderichsen syndrome is a pathology resulting from acute hemorrhagic infarction of both adrenal glands ( that is, a massive hemorrhage into the tissues of the adrenal glands) and commonly seen in sepsis ( severe, systemic, inflammatory condition caused by the spread of infection throughout the body from the primary focus). With sepsis, DIC often develops, which, in fact, serves as the direct cause of hemorrhage into the adrenal glands. With such internal bleeding, the tissues of the adrenal glands quickly overflow with blood. The blood flow in them slows down sharply, venous blood from the adrenal glands is not removed, and new arterial blood does not enter in sufficient quantities. Because of this, the cells of the adrenal cortex quickly die. Adrenal insufficiency develops. The rapid overflow of the adrenal glands with blood in the Waterhouse-Friderichsen syndrome is provoked not only by a violation of blood clotting ( caused by DIC), but also by the peculiarities of the blood supply of the adrenal glands themselves.
Most often, Waterhouse-Frideriksen syndrome occurs with sepsis that develops against a background of meningococcal infection.

The pathogenesis of Addison's disease

pathogenesis ( development mechanism) Addison's disease is caused by a deficiency in the body of aldosterone, cortisol and excess secretion of melanocyte-stimulating hormone. Aldosterone is an important hormone that regulates the body's sodium and water levels, the amount of potassium and hemodynamic status. It acts on the kidneys and causes them to retain sodium and water in the body ( in exchange for potassium), thus preventing their rapid removal from the body. With aldosterone deficiency, the kidneys begin to quickly excrete sodium and water in the urine, which leads to rapid dehydration of the body, disruption of the water and electrolyte balance in the body, thickening of the blood, slowing down of blood circulation and blood supply to peripheral tissues. Against the background of these changes, the cardiovascular system is most seriously affected ( increased heart rate, heart pain, decreased blood pressure, etc.), gastrointestinal tract ( abdominal pain, nausea, constipation, vomiting, etc.), central nervous system ( fainting, convulsions, mental disorders, headache, etc.). Violation of the excretion of potassium from the body during primary hypocorticism contributes to its accumulation in the blood and the development of the so-called hyperkalemia, which has a harmful effect on the functioning of the heart and skeletal muscles.

The absence of cortisol in the body makes it very sensitive to stress factors for it ( for example, infections, physical overexertion, mechanical injuries, etc.), under the action of which, in fact, the decompensation of Addison's disease occurs.

Cortisol is one of the hormones that regulate carbohydrate metabolism in the body. It stimulates the formation of glucose from other chemicals ( gluconeogenesis), glycolysis ( glycogen breakdown). In addition, cortisol has a similar effect on the kidneys as aldosterone ( that is, it contributes to the retention of water and sodium in the body and the removal of potassium). This hormone also affects protein and fat metabolism, increasing the breakdown of proteins and the accumulation of fats in peripheral tissues. Cortisol interacts well with thyroid hormones and catecholamines, that is, the hormones of the adrenal medulla. Cortisol deficiency in Addison's disease leads to a violation of carbohydrate, protein, fat metabolism and a decrease in overall resistance, that is, the body's resistance to stress.

Patients with Addison's disease often have hyperpigmentation of the skin ( increased deposition of melanin pigment in the skin). It is caused by an excess of melanocyte-stimulating hormone in the blood, which stimulates melanocytes ( pigment cells) skin to the production of melanin. Such an excess occurs due to the fact that during primary hypocorticism in the pituitary gland, the concentration of the precursor common to melanocyte-stimulating and adrenocorticotropic hormones, proopiomelanocortin, increases. Proopiomelanocortin has no hormonal properties. According to its chemical structure, it is a large protein molecule, which, when split ( certain enzymes) is divided into several hormone peptides ( adrenocorticotropic, melanocyte-stimulating, beta-lipotropic hormones, etc.). Increased levels of melanocyte-stimulating hormone in the blood gradually cause darkening of the skin, which is why Addison's disease is also called bronze disease.

Symptoms and signs of Addison's disease

Patients with Addison's disease, when contacting a doctor, most often complain of their general weakness, increased fatigue, chronic fatigue, recurrent headache, dizziness, and fainting. They often experience anxiety, anxiety, increased internal stress. In some patients, neurological and psychiatric disorders can be detected ( memory impairment, decreased motivation, negativism, loss of interest in ongoing events, increased irritability, depression, depression, impoverishment of thinking, etc.). All these symptoms are due to disorders in all types of metabolism ( carbohydrate, protein, lipid, water-electrolyte) in the brain.

In primary hypocorticism, patients experience progressive weight loss. This is due to the constant loss of fluid by the body, the presence of anorexia in them ( lack of appetite) and a true decrease in the amount of muscle mass. They often have muscle weakness, myalgia ( muscle pain), muscle cramps, tremors ( ), loss of sensation in the extremities. Muscular symptoms can be explained by disturbances in water and electrolyte balance ( in particular, increased levels of potassium and calcium in the blood) that are commonly seen in these patients.

Quite common in Addison's disease is a variety of gastrointestinal ( gastrointestinal) symptoms in the form of nausea, vomiting, constipation ( sometimes diarrhea), diffuse ( common) abdominal pain. Patients are constantly craving salty foods. In addition, they are constantly worried about the feeling of thirst, so they often drink water. Often, such patients are observed for a long time by a gastroenterologist due to the fact that they have various diseases of the gastrointestinal system ( e.g. stomach ulcer, duodenal ulcer, spastic colitis, gastritis), and adrenal insufficiency remains undetected for a long time. Gastrointestinal symptoms in Addison's disease tend to correlate with the degree of anorexia ( lack of appetite) and weight loss.

One of the main symptoms of Addison's disease is hypotension ( low blood pressure). In most patients, systolic blood pressure ranges from 110 to 90 mmHg, and diastolic blood pressure may fall below 70 mmHg. In the initial stages of the disease, hypotension can only be orthostatic in nature ( that is, blood pressure will fall when changing body position from horizontal to vertical). In the future, arterial hypotension begins to occur when the body is exposed to almost any stress factor for a person. In addition to low blood pressure, tachycardia may also occur ( cardiopalmus), pain in the heart, shortness of breath. Sometimes blood pressure may remain within normal limits, in rare cases it may be elevated ( especially in patients with hypertension). In women suffering from Addison's disease, the menstrual cycle is sometimes disturbed. Periods either disappear completely ( i.e. amenorrhea) or become irregular. This often seriously affects their reproductive function and results in various problems associated with pregnancy. In men with this disease, impotence appears ( erectile dysfunction of the penis).

Hyperpigmentation in Addison's disease

Hyperpigmentation ( skin darkening) in Addison's disease is one of the most important symptoms characteristic of this pathology. There is usually a clear correlation ( addiction) between the duration of the presence of adrenal insufficiency in a patient, its severity and the intensity of the color of the skin and mucous membranes, since this symptom occurs in patients one of the first. In most cases, exposed areas of the body that are constantly in contact with sunlight begin to darken first. Most often they are the skin of the hands, face and neck. In addition, there is an increase in pigmentation ( melanin deposits) in the area of ​​those parts of the body that normally have a fairly intense dark color. We are talking about the skin of the scrotum, nipples, perianal zone ( skin around the anus). Then the skin of the folds on the palms begins to darken ( palmar lines), as well as those areas on which folds of clothing constantly rub ( this is observed in the area of ​​the collar, belt, elbow bends, etc.).

In some cases, in some patients, the mucous membrane of the gums, lips, cheeks, soft and hard palate may darken. In later stages, diffuse hyperpigmentation of the skin is noted, which can have a different severity. The skin can acquire a smoky, bronze, brown tint. If Addison's disease is caused by autoimmune destruction of the adrenal cortex, then often against the background of pigmentation on the skin, patients may develop vitiligo ( pigmentless, white patches). Vitiligo can occur almost anywhere on the body and come in a variety of sizes and shapes. It is easy enough to detect, since the skin of such patients is much darker than the spots themselves, which creates a clear contrast between them. Very rarely, skin hyperpigmentation may be absent or minimal in Addison's disease ( inconspicuous), this condition is called "white addisonism". Therefore, the absence of darkening of the skin is not yet proof that this patient does not have adrenal insufficiency.

Diagnosis of Addison's disease

Diagnosis of Addison's disease is currently not a difficult task. The diagnosis of this pathology is made on the basis of clinical ( history taking, physical examination), laboratory and radiation research methods. At the first stage of diagnosis, it is important for the doctor to understand whether the symptoms are in fact ( for example, hyperpigmentation of the skin, low blood pressure, general weakness, abdominal pain, nausea, vomiting, menstrual irregularities, etc.), detected ( through clinical examinations) in the patient who applied to him, signs of primary adrenal insufficiency. To confirm this, he assigns him the passage of some laboratory tests ( complete blood count and biochemical blood test, urinalysis). These studies should reveal the presence in the patient in the urine and in the blood plasma of a reduced level of adrenal hormones ( aldosterone, cortisol), increased concentration of adrenocorticotropic hormone ( ACTH), renin, as well as a disturbed state of the water-electrolyte state of the blood, carbohydrate metabolism and certain disorders in the cellular composition of the blood.

In addition, in addition to laboratory tests, short-term and long-term diagnostic tests are prescribed to confirm primary hypocorticism. These tests are stimulation. The patient is injected intramuscularly or intravenously with artificially synthesized adrenocorticotropic hormone ( ACTH), which is produced under the commercial name of synacthen, synacthen-depot or zinc-corticotropin. Normally, when ingested, these drugs should stimulate the adrenal cortex, as a result of which its tissues will begin to intensively secrete hormones ( cortisol), the concentration of which is determined in the blood 30 and 60 minutes after the administration of the drugs. If the patient is ill with Addison's disease, then the adrenal glands will not be able to adequately respond to stimulation with synacthen ( or synacthenom depot, or zinc corticotropin), as a result of which the level of adrenal hormones in the blood plasma will remain unchanged.

After confirming the presence of Addison's disease in a patient, it is important for the doctor to establish its etiology. Because in the vast majority of cases ( in 80 - 90%) the cause of this endocrine disease is an autoimmune lesion of the tissues of the adrenal cortex, then the patient needs to donate blood for an immunological study. With it, it will be possible to detect antibodies to the enzymes of adrenal steroidogenesis ( the formation of steroid hormones)-21-hydroxylase ( P450c21), 17a-hydroxylase ( P450c17), side chain cleavage enzyme ( P450scc), which are markers ( indicators) primary autoimmune hypocorticism. If such antibodies were not detected, then the next diagnostic test to be prescribed is a biochemical blood test for the content of long-chain fatty acids in it ( JK).

Identification of JAD is an important diagnostic sign of adrenoleukodystrophy, which is the third most common cause of Addison's disease. In the case of a negative result for the presence of JFA in the blood, the patient is usually prescribed the passage of radiation studies ( computed tomography, magnetic resonance imaging) needed to visualize the structure of the tissues of the adrenal cortex. These studies, as a rule, confirm the presence of cancer metastases or a tuberculous focus in the adrenal glands.

Diagnostic measures for DIC, Waterhouse-Friderichsen syndrome, antiphospholipid syndrome, fungal infections, HIV infection are quite difficult to consider, since there are many factors that influence the choice of one or another diagnostic method. It all depends on the specific situation. In addition, all these pathologies are quite rare causes of Addison's disease, and it is not always so easy to suspect the presence of adrenal insufficiency in them.

Diagnostic methods used to detect Addison's disease

Method name	Why carry out this study?
Anamnesis	When collecting an anamnesis, the doctor asks the patient about the complaints that bother him and about the conditions that contributed to their appearance. The main symptoms of Addison's disease are fatigue, general weakness, dizziness, headache, fainting, restlessness, anxiety, thirst, craving for salty foods, muscle weakness, myalgia ( muscle pain), muscle cramps, abdominal pain, nausea, vomiting, menstrual irregularities, etc.
Visual inspection	An external examination of patients suffering from Addison's disease can reveal increased skin pigmentation, vitiligo, weight loss, arterial hypotension ( low blood pressure), various mental disorders ( memory impairment, decreased motivation, loss of interest in ongoing events, increased irritability, depression, depression, etc.), tremor ( involuntary trembling of fingers), loss of sensation in the extremities.
Radiography and computed tomography	CT scan ( CT) and magnetic resonance imaging ( MRI) are used to detect metastases, tuberculous foci in the adrenal glands. Radiography is usually prescribed to detect tuberculosis in the lungs. CT and MRI of the brain and spinal cord are prescribed to confirm the diagnosis of adrenoleukodystrophy.
Magnetic resonance imaging
General blood analysis	In Addison's disease, anemia can be detected in the general blood test ( decrease in the number of red blood cells and hemoglobin), lymphocytosis ( an increase in the number of lymphocytes), neutropenia ( decrease in the number of neutrophils), eosinophilia ( an increase in the number of eosinophils), elevated ESR ( erythrocyte sedimentation rate) .
Blood chemistry	A biochemical blood test can detect hypoglycemia ( low glucose), hyponatremia ( lower sodium levels), hypochloremia ( chlorine reduction), hypercalcemia ( high calcium content), hyperkalemia ( high potassium content), reduced levels of cortisol, aldosterone, increased concentration of adrenocorticotropic hormone ( ACTH), renin. If a doctor suspects adrenoleukodystrophy in a patient as the cause of Addison's disease, then he may also prescribe an analysis for the content of long-chain fatty acids in the blood ( JK).
Immunological blood test	An immunological blood test is ordered to confirm autoimmune genesis ( origin) adrenal insufficiency. In such cases, the patient should be tested for blood levels of antibodies to adrenal steroidogenesis enzymes ( the formation of steroid hormones)-21-hydroxylase ( P450c21), 17a-hydroxylase ( P450c17), side chain cleavage enzyme ( P450scc). Such a study can also detect antibodies to the human immunodeficiency virus ( HIV) and systemic fungal infections, which may be one of the causes of Addison's disease. In addition, an immunological test is often prescribed for suspected antiphospholipid syndrome, in which anticardiolipin antibodies, antibodies to B-2-glycoprotein 1, and lupus anticoagulant can be detected in blood plasma ( VA).
General and biochemical analysis of urine	Urinalysis can reveal an increase in its daily volume, hypostenuria ( decrease in the relative density of urine), elevated sodium, reduced aldosterone, 17-hydroxycorticosteroids ( total content of various types of glucocorticoids produced in the adrenal cortex), as well as 17-ketosteroids ( androgens produced by the adrenal cortex).
Diagnostic tests	Short-term ( with synacthen) and long ( with synacthen depot or zinc corticotropin) diagnostic tests for Addison's disease reveal a significant decrease in the secretion of hormones by the adrenal cortex during their stimulation.
Biopsy of adrenal tissue	Biopsy ( microsurgical operation aimed at taking a piece of tissue for cytological examination) adrenal tissue is extremely rare. In most cases, it is done to clarify the nature of the damage to the tissues of the adrenal cortex.
General sputum analysis	A general sputum analysis is prescribed in cases where the doctor suspects a tuberculous lesion of the patient's adrenal cortex.

Treatment of Addison's disease

For the treatment of Addison's disease, lifelong drug therapy is prescribed, which allows you to constantly replenish the deficiency of adrenal hormones ( corticosteroids). For this purpose, patients are prescribed hormone replacement drugs, which are of two types. The first type of hormonal preparations includes medicines that correct the balance of mineralocorticoids in the body. The second type of hormone prescribed for Addison's disease includes drugs that increase the level of glucocorticoids in the blood. Both types of hormones must always be combined and used in parallel.
Cortineff is usually prescribed as a replacement mineralocorticoid ( or fludrocortisone). Should be drunk in the morning once a day). The daily dose, as a rule, is selected individually and always ranges from 0.05 to 0.2 mg. The adequacy of the appointment of Cortineff should be constantly under clinical and laboratory control.

Patients receiving drug treatment with this hormonal drug should have normal blood pressure, potassium, sodium, and renin concentrations ( a specific enzyme produced by the kidneys) in blood plasma. In addition, they should not show signs of puffiness ( e.g. swelling in the legs, arms, face), as it will indicate fluid retention in the body, which will serve as an indirect sign of an overdose of Cortineff. The selection of the dosage of this drug during pregnancy is carried out taking into account the level of potassium in the blood and blood pressure. In this case, the dosage often has to be increased, due to the fact that certain hormonal changes occur in the pregnant woman's body.

Replacement mineralocorticoids in Addison's disease should definitely be prescribed in combination with synthetic glucocorticoids. Depending on the duration of their action, the latter are divided into three groups ( short, medium and long acting). Short acting glucocorticoids hydrocortisone, cortisone) can be assigned either two ( morning and afternoon), or three ( morning, afternoon and evening) times a day. Intermediate-acting drugs ( prednisolone) usually need to be consumed twice a day ( morning and afternoon). A third type of glucocorticoid ( dexamethasone), which have a long duration of action and are not excreted from the body for a long time, it is recommended to drink once a day ( morning or late evening). All types of hormonal medications should be used after meals.

Therapeutic regimens for prescribing glucocorticoids for the treatment of Addison's disease

Group of glucocorticoids	Name of the drug	Time of receipt	Dosage
(three-time scheme)	Hydrocortisone	In the morning	15 - 20 mg
		After lunch	5 - 10 mg
		In the evening	5 mg
	Cortisone	In the morning	25 mg
		After lunch	12.5 mg
		In the evening	6.25 mg
Short acting glucocorticoids (two-time scheme)	Hydrocortisone	In the morning	20 mg
		After lunch	10 mg
	Cortisone	In the morning	25 mg
		After lunch	12.5 mg
Intermediate acting glucocorticoids	Prednisolone	In the morning	5 mg
		After lunch	2.5 mg
Long acting glucocorticoids	Dexamethasone	Morning or late evening	0.5 mg

As in the case of mineralocorticoids, the effectiveness of drug therapy with synthetic glucocorticoids ( hydrocortisone, prednisolone, dexamethasone, cortisone) should also be closely monitored. With proper treatment, the patient should disappear the main signs of Addison's disease - general weakness, fatigue, chronic fatigue, recurrent headaches, dizziness, hunger, arterial hypotension ( reduced blood pressure), hyperpigmentation on the skin, neurological and mental disorders ( memory impairment, decreased motivation, loss of interest in ongoing events, increased irritability, depression, depression, etc.) and etc.

After the start of treatment, body weight usually begins to return immediately, and excessive weight gain should not occur ( this would be a sign of a glucocorticoid overdose). Gastrointestinal ( gastrointestinal) symptoms on the background of hormone replacement therapy with mineralocorticoids and glucocorticoids should also disappear fairly quickly. It should be remembered that hormonal drugs that are prescribed for adrenal insufficiency have a sufficient number of side effects and contraindications, so you must strictly adhere to that course of treatment ( and those dosages of drugs) as prescribed by the doctor.

If it has been determined that Addison's disease was caused by a tuberculosis infection, then the patient should be given additional treatment, which should include antibiotics ( rifampicin, streptomycin, isoniazid, ethambutol, etc.). Antibacterial agents for tuberculosis are prescribed according to special therapeutic regimens and are selected individually. With fungal infection of the adrenal cortex, systemic antifungal drugs are prescribed ( e.g. ketoconazole, fluconazole, etc.). In antiphospholipid syndrome, in addition to hormonal therapy, anticoagulants of indirect action are prescribed ( warfarin, acenocoumarol, etc.) and antiplatelet agents ( aspirin). The choice of treatment tactics for DIC and Waterhouse-Friderichsen syndrome always depends on their etiology, severity and presence of complications. With a metastatic lesion of the adrenal cortex, surgery, as a rule, will not give anything useful to the patient, so he is treated only with hormone replacement therapy.

Mineralocorticoids and glucocorticoids should not be taken in tablet form if the patient has a gastrointestinal disorder. In such cases, it is better to administer the drug as an injection ( parenterally). The fact is that disturbances in the gastrointestinal system can seriously affect the absorption of hormonal drugs into the blood, as a result of which the normal daily dosing regimen will be spoiled. If a patient with Addison's disease needs elective surgery for any reason ( for example, removal of the appendix, gallbladder, hernia, etc.), then on the days before surgery, he should be given a course of hydrocortisone injections of 50 mg every 8 hours. Immediately before the operation, he needs to inject 75 - 100 mg of hydrocortisone intramuscularly. During the operation, he should inject 75 - 100 mg of this drug diluted in a glucose solution.

After surgery, the first four days the patient needs to inject hydrocortisone 50 mg every 6 to 8 hours. On the 5th - 6th day, the dose of hydrocortisone is reduced. At this time, you should enter 50 mg 2 times a day and use prednisone in tablet form, 5-10 mg 2-3 times a day. From 7 to 8 days, the patient is transferred to the usual regimen of therapeutic treatment with glucocorticoids, which should already be taken in full in the form of tablets. The dosage of mineralocorticoids is selected individually. Such a drug therapeutic regimen in the preoperative, operational and postoperative periods is necessary in order to prepare the patient's body for severe stress, which is surgery.

Diet for Addison's disease

The diet for Addison's disease should be aimed at replenishing the deficiency of sodium and fluid in the patient's body, as well as restoring his carbohydrate, protein and fat metabolism. In a day, such patients are shown the use of an increased amount of table salt ( 20 - 25 g) and water. Due to the fact that a significant amount of potassium is retained in their body, they are also recommended to consume less products ( e.g. coffee, tea, cocoa, raisins, spinach, nuts, mushrooms, etc.) rich in this chemical element. In order to restore metabolism ( metabolism) such people need to consume more food rich in vitamins of groups A, B, C, D.
Their diet should have a high energy value, which will soon ( with the right medication) will help them quickly gain normal weight.

Patients with Addison's disease should consume an increased amount of protein per day ( 1.5 g/kg body weight), carbohydrates ( 450 - 500 g/day) and fats ( 120 - 130 g/day). The diet should be fractional. It is worth eating 4-5 times a day in small portions. The diet must include foods that stimulate appetite and gastrointestinal secretion ( e.g. meat broths, sauces, pickled vegetables, eggs, etc.), which are quite often violated in such patients.

Foods that are indicated and contraindicated in Addison's disease

Examples of Foods to Eat for Addison's Disease

Examples of foods that are not desirable to consume with Addison's disease

broths ( meat and fish); pickled or pickled vegetables; stew or fish; sauces ( meat, fish, vegetable); salted or smoked meat or fish products; eggs ( fried or boiled); canned food ( meat, fish and vegetable); milk products ( milk, butter, cottage cheese, sour cream, feta cheese, cheese, etc.); jam; fruits and berries ( apples, oranges, grapes, pears, strawberries, grapefruit); fruit and vegetable juices; cereals ( semolina, wheat, rice, barley).

beverages ( coffee, tea, cocoa, alcohol, carbonated drinks); raisin; nuts; vegetables ( spinach, Brussels sprouts, kohlrabi, beets); mushrooms; cereals ( buckwheat, oatmeal); peaches; chocolate; dried apricots; legumes ( peas, soybeans, lentils).

What is an Addisonian crisis?

Addisonian crisis ( acute adrenal insufficiency) is a life-threatening condition that can occur with Addison's disease. Addisonian crisis can occur in a patient in various stressful situations ( for example, during surgery, in case of severe mechanical injury, infectious disease, physical overstrain, etc.). Such a crisis is not uncommon in patients who receive inadequate hormone replacement therapy. In addition, acute adrenal insufficiency can occur when Addison's disease has not yet been diagnosed in a patient. Addisonian crisis is characterized by various symptoms. Its main symptoms are indomitable vomiting, nausea, aversion to food, diarrhea, a sharp decrease in blood pressure, a significant slowdown in the pulse, increased pigmentation on the skin, severe pain in the abdomen, legs, back pain, loss of consciousness, mental and neurological disorders ( lethargy, delirium, convulsions, stupor, hallucinations, etc.) and etc.

If a patient develops an Addisonian crisis, they should be hospitalized immediately. Treatment in such situations is reduced to a massive rehydration ( that is, an intravenous infusion of liquid to the patient), hormonal and etiotropic therapy. Glucose solution is usually used as rehydration solutions. Hormone therapy involves the intravenous administration of large doses of hydrocortisone to the patient ( 100 mg) several times a day. Further, the dose of this drug is gradually reduced. At such high doses of hydrocortisone, the patient does not need to be prescribed mineralocorticoids ( cortineff). The latter most often begin to prescribe to the patient at the moment when the daily dosage of glucocorticoids drops below 100 mg / day. When the patient's condition stabilizes, he is smoothly transferred to drugs in tablet form. Etiotropic therapy prescribed for the Addisonian crisis is required to eliminate the very cause that could cause it. In most cases, antibiotics are used as such therapy ( in the event that the Addisonian crisis was caused by an infectious disease).

How does Addison's disease manifest itself in women?

Addison's disease in women manifests itself in the same way as in men. All patients with this pathology have cardiovascular ( decreased blood pressure, palpitations, pain in the heart, shortness of breath), gastrointestinal ( nausea, vomiting, diarrhea, lack of appetite, abdominal pain, craving for salty foods, intense thirst), dermatological ( vitiligo, hyperpigmentation of the skin) symptoms and signs. In women with Addison's disease, body weight decreases, they periodically feel increased internal tension, fear, anxiety, and anxiety.
They constantly have general weakness, increased fatigue, reduced performance. They often find it difficult to get out of bed in the morning.

In most patients, one can observe a depressed state, lack of initiative, increased irritability, memory impairment, and indifference to what is happening around. They often complain about the presence of muscle weakness, myalgia ( soreness in the muscles), tremor ( involuntary trembling of fingers), violations of the sensitivity of the skin of certain areas on the lower and upper extremities. In women suffering from this disease, some irregularities in the dynamics of the menstrual cycle can sometimes be detected. Menstruation, in some cases, may simply not occur ( i.e. amenorrhea) or be irregular and untimely, which sometimes affects reproductive function and results in problems associated with conceiving a child.

How are Itsenko-Cushing's and Addison's diseases related?

Itsenko-Cushing's disease is an endocrine disease in which the adrenal glands produce excessive amounts of glucocorticosteroids. This condition is also called hypercortisolism. The mechanism of development of this disease is associated with a violation of adequate secretion of adrenocorticotropic hormone by the pituitary gland of the brain ( ACTH), which normally should control the blood levels of glucocorticosteroids secreted by the adrenal cortex. In Itsenko-Cushing's disease, patients have a constant, uncontrolled production of large amounts of ACTH and their release into the blood.

Under conditions of high concentration of adrenocorticotropic hormone in the bloodstream, the adrenal glands begin to function more intensively, which is accompanied by a periodic release of new portions of glucocorticosteroids into the blood ( cortisol). One of the methods for the treatment of Itsenko-Cushing's disease is bilateral adrenalectomy ( removal of both adrenal glands), after which adrenal insufficiency always develops ( Addison's disease).

Is there secondary Addison's disease?

There is no secondary Addison's disease. In medicine, there is a so-called secondary hypocorticism ( or secondary adrenal insufficiency). Addison's disease primary hypocorticism or primary adrenal insufficiency) occurs when the adrenal cortex is damaged by some harmful factor, as a result of which its cells die and stop synthesizing and secreting hormones into the blood ( mineralocorticoids, glucocorticosteroids and androgens). With secondary hypocorticism, the tissues of the adrenal cortex are not damaged, they remain healthy. With this pathology, the production of adrenocorticotropic hormone (ADH) is disrupted ( ACTH) in the pituitary gland, which is located in the brain. Through this hormone, the pituitary gland regulates the secretion of glucocorticosteroids and androgens into the blood by the adrenal cortex.
Adrenocorticotropic hormone has a direct stimulating effect on fascicular cells ( Where are glucocorticosteroids formed?) and mesh ( where are androgens formed) zones of the adrenal cortex. ACTH has little to no effect on the production of mineralocorticoids by the adrenal glands ( aldosterone).

In secondary hypocorticism, the pituitary gland produces little adrenocorticotropic hormone (ADH). or stop making it altogether.), as a result of which the tissues of the adrenal glands, dependent on this hormone, cease to function normally. This is how secondary adrenal insufficiency develops. Such deficiency differs from Addison's disease not only in pathogenesis ( origin of the disease), but also by clinical symptoms, as well as by some laboratory parameters. These differences are used by doctors in the differential diagnosis of these two pathologies.

Differential diagnosis of primary and secondary hypocorticism

sign	Primary hypocorticism ( Addison's disease)	Secondary hypocorticism
Aldosterone level in the blood	Short	Fine
The level of cortisol in the blood	Short	Short
Adrenocorticotropic hormone level(ACTH)in blood	High	Short
Hyperpigmentation of the skin and mucous membranes	Present	Missing
Arterial hypotension	strongly expressed	Moderate or mild
General weakness	strongly expressed	Moderate or mild
Functional test with synacthen	Negative ( adrenal glands do not respond to stimulus)	Positive ( adrenal glands respond to stimulus)
Gastrointestinal symptoms	strongly expressed	Moderate or mild
Thirst	strongly expressed	None or mild
cravings for salty foods	strongly pronounced	None or mild
Weight loss	strongly pronounced	strongly pronounced