Addison's disease (aka gipokortitsizm) - is a group of clinical symptoms caused by chronic diseases of the thyroid of the adrenal cortex, resulting in inappropriate secretion of aldosterone and cortisol.

The adrenal glands are the organ that produces hormones. They consist of a core generating a stress hormone - adrenaline and the outer cortex produces steroid hormones, including cortisol and aldosterone have important functions in the body. Cortisol is involved in the metabolism of proteins, fats and sugars. Aldosterone is involved in the regulation of blood pressure through its influence on the sodium concentration in the body. Lack or absence of these hormones lead to severe metabolic disorders, which, if run, will inevitably lead to the death of the patient. Today's medicine allows you to fully make up for the missing hormones in the blood, so patients can live a normal life.



The cause of Addison's disease can be any other disease, leading to irreversible damage to the adrenal cortex. The disease occurs in about 5-10 people per 100 000. There are primary adrenocortical insufficiency, in which damaged only the adrenal glands, and secondary failure, when the system comes out of the pituitary gland, which regulates the work of the adrenal glands.

Primary adrenal insufficiency

Primary adrenal insufficiency (Addison's disease classic) is the result of the destruction of both adrenal glands. The most common cause of damage to adrenal responsible for 90% of all cases, is the so-called autoimmune reaction. This is a disease in which the patient's immune system begins to attack its own tissues. If the immune system attacks the adrenal cortex, which leads to its failure. The adrenal cortex is exposed in the first phase, an increase due to the occurrence of edema limfotsitovyh. She starts to lose its properties and ceases to act as the delivery of hormones in the body in sufficient quantity. This can lead to hormonal imbalance and the appearance of the first symptoms of Addison's disease. At the second stage the adrenal glands are reduced or even subjected to gradual disappearance. They stop producing cortisol, and it becomes necessary to its continuous introduction.

Another cause of Addison's disease is a tumor. If the cancer takes both adrenal glands, this may impair the function that will manifest symptoms of Addison's disease. This occurs most often as a result of metastatic kidney cancer, breast and lung. To this can also cause an initial adrenal cancer, lymphoma usually present simultaneously on the two adrenal glands. In this case, the main concern should be the fight against the original cause (cancer).

Congenital adrenal hyperplasia can also cause a deficiency of cortisol in the blood. This disease is inherited genetically. Adrenal hyperplasia is associated with defective synthesis of cortisol in the brain by stimulating the adrenal glands to work more hard. Those unable to secrete cortisol, secrete more androgens than necessary. As a result of sharply increased secretion of androgens while cortisol deficiency. This disease, in addition to standartynh symptoms of adrenal insufficiency, can cause a number of additional changes in the clinical picture. We were born girls can be the cause hermaphroditism. Women often leads to polycystic ovary syndrome, infertility and metabolic syndrome. In men may impair the functioning of the testes, and hence infertility.

Another disorder, leading to the development of Addison's disease - is adrenoleukodystrophy (also known as Schilder's disease). The cause of this disease is ABCD1 gene mutation responsible for lipid utilization. This damage leads to the accumulation of long chain fatty acids. These acids promote nerve damage of the myelin sheath in the central nervous system and the adverse changes in the adrenal cortex. Illness gives both neurological and typical symptoms of Addison's disease. Neurological symptoms - a muscular tremor, ataxia, disturbances of perception and hearing, as well as seizures. The first symptoms usually appear in children. There is sometimes a very acute form, when the first symptoms appear in infancy: the child is poorly developed and, as a rule, dies at the age of ten. This disease is incurable. Currently under development of gene therapy techniques. Also investigated the effectiveness of appropriate diet, low in fatty acids with a long chain.

Another rare and congenital disease in which there is a disruption of the adrenal cortex, Olgrouva syndrome (AAA). This disease is inherited in an autosomal recessive manner (parents must transmit defective genes). People with this syndrome have problems with the release of tears that causes dry eyeballs. Another common symptom is esophageal achalasia or its abbreviation, which interferes with normal digestion.

Temporary reduction in cortisol production can also be caused by taking medication. Some antifungal drugs, cancer, and other steroids can lead to a reduction in cortisol production, and produce symptoms characteristic of Addison's disease. After drug withdrawal symptoms of Addison's disease are retreating, and the adrenal glands return to their normal functioning.

The last group of factors contributing to the occurrence of the primary hypofunction of the adrenal cortex - infection. Bacterial (tuberculosis, bleeding in the adrenal gland during sepsis), fungal (histoplasmosis, cryptococcosis, coccidioidomycosis, blastomycosis) and viral infections (infections that accompany AIDS) may cause temporary or permanent damage to the adrenal cortex. Treatment is reduced to the fight against the main disease and prevention of its complications.

Secondary adrenal insufficiency

Secondary insufficiency of the adrenal cortex is associated with dysfunction of the pituitary gland. The pituitary gland, in particular, highlights adrenocorticotropic hormone that stimulates secretion of cortisol from the adrenal cortex. If for any reason there is a deficiency of adrenocorticotropic hormone, and reduces the secretion of adrenal cortex hormones. And it falls mainly cortisol production, and aldosterone levels may be within normal limits. Isolation of adrenocorticotropic hormone closely related to the level of cortisol in the blood - higher levels of cortisol, the lower the secretion of adrenocorticotropic hormone, and vice versa. This is called a negative feedback, which results in a stable balance of a healthy person. In people with pituitary dysfunction levels of adrenocorticotropic hormone does not meet the rise in cortisol levels decrease.

Secondary adrenal insufficiency may also be caused by hypophysis diseases caused usually autoimmunity. This time, however, it is the pituitary gland becomes the target of the attack of the immune system and the cortisol deficiency develops again. Another reason is the pituitary tumor. The pituitary gland can also be damaged during a stroke or injury caused by external factors.

One of the common causes of secondary adrenal insufficiency is the so-called Sheehan's syndrome. In this disease there is necrosis of the pituitary gland, caused by intracranial hemorrhage and hypovolemic shock during delivery. During the development of the fetus in the mother increases hypertrophy of the pituitary gland, and, moreover, deteriorates its blood supply. If during childbirth, a large blood loss occurs, it can damage the pituitary region, which leads to irreversible damage and shock function hormone secretion.

Specific Sheehan syndrome symptoms - the sudden appearance of disorder (lack of pituitary hormones, lack of lactation, absence of menstrual cycles, decreased libido and symptoms of secondary deficiency of adrenal hormones) soon after birth.

The symptoms of Addison's disease

Chronic adrenal insufficiency is characterized by:

• the general weakening of the muscles;
• inability to great physical stress;
• tendency to fainting;
• gray-colored coloring skin and mucous membranes, particularly on the back lines and folding hands, elbows, and other areas exposed to sunlight. Brown traces associated with too high levels of pituitary hormone - ACTH;
• darkening of nipples, freckles and scars;
• lower blood pressure;
• disturbances in the metabolism and action of gonads;
• hair loss;
• constant feeling of cold;
• poor tolerance of stressful situations;
• changes in mood and behavior (depression, anxiety);
• nausea, vomiting, impaired excretion stool, diarrhea, abdominal pain,
• lack of appetite, emaciation, excessive appetite for salt.

The severity of symptoms depends on the amount of hormones produced. Symptoms of the disease will be amplified in the case of any injuries, infections, pregnancy and childbirth. The disease can lead to the so-called crisis of the adrenal glands - acute adrenal insufficiency, causing sudden appearance of clinical symptoms. It is manifested initially weakening, loss of appetite, diarrhea, vomiting, fever or low body temperature, and then a sharp drop in blood pressure and fainting. adrenal crisis develops on the background of chronic adrenal cortex hypofunction when a person suffering from this disorder do not accept synthetic analogs of cortisol and facing strong stress level of the situation, infection or other debilitating factor. Great stress is very high momentary demand for cortisol, and develop a strong external symptoms in the absence or insufficient quantity.

The second group of people, particularly prone to adrenal crisis, are patients with undiagnosed Addison's disease who do not take drugs at all. The third group consists of people who have a sudden damage to the adrenal glands, as a result of mechanical trauma or as a result of bleeding during sepsis. In the case of adrenal crisis in the absence of appropriate treatment, the patient is at risk of dying, so it is important to as quickly as possible the provision of appropriate drugs (hydrocortisone, physiological saline, glucose) and treatment of infections that cause this condition.