Addison's disease is a primary adrenal insufficiency (PAI), which is caused by the destruction of the glandular tissue of the adrenal glands and a decrease in hormone synthesis. The disease occurs against the background of autoimmune pathologies, damage to the endocrine glands by the tuberculous process. Classic symptoms include hyperpigmentation of the skin, general and muscular weakness, dyspeptic disorders. Immunological and hormonal blood tests, methods of radiation diagnostics are used for diagnosis. Treatment of Addison's disease includes hormone replacement therapy, diet therapy, correction of the root cause of pathology.
The clinical syndrome associated with adrenal gland damage was first described by the English therapist Thomas Addison in 1855. Later, the connection between the characteristic symptom complex and adrenal insufficiency was confirmed by the French doctor A. Trousseau, who called it "Addison's disease". In practical endocrinology, the disease occurs with a frequency of 35 to 140 cases per 1 million population. The peak of diagnosis occurs at the age of 20-50 years, pathology develops more often in women.
Autoimmune damage to the adrenal glands, which accounts for up to 90% of all causes of the disease, is recognized as the main trigger factor of Addison's disease. Adrenal insufficiency is often accompanied by other autoimmune processes: vitiligo, type 1 diabetes mellitus, hypothyroidism and thyroiditis. With a combination of such pathologies, polyglandular syndrome is diagnosed. Other causes of adrenal insufficiency:
All of the above causes cause primary adrenal insufficiency, which has a similar pathogenesis and clinical picture. In order to avoid confusion in terminology, endocrinologists currently use the name "Addison's disease" only to refer to PAI of autoimmune and tuberculosis genesis. It is these variants of the disease that Thomas Addison described in his works.
The adrenal glands are a paired endocrine organ that consists of cortical and medullary matter. A number of hormones are produced in the cortical layer: mineralocorticoids (aldosterone), glucocorticoids (cortisol), sex steroids (androgens and estrogens). The mechanism of development of Addison's disease is associated with an insufficient amount of aldosterone and cortisol, which are involved in metabolic processes and affect almost all internal organs.
With a deficiency of cortisol, the main adaptogenic hormone, there is a decrease in resistance to endo- and exogenous stress factors, in which the normal concentration of glucocorticoids increases tenfold. In addition, patients develop circulatory insufficiency, dehydration, inhibition of gluconeogenesis and a decrease in glycogen stores in the liver.
The lack of aldosterone causes an increase in potassium levels and a decrease in the amount of sodium in the blood. With a shortage of this hormone, increased water losses occur through the kidneys and gastrointestinal tract, resulting in a decrease in the volume of circulating blood and arterial hypotension develops. Excess potassium disrupts the heart, worsens intraventricular conduction.
The clinical picture develops gradually, often patients cannot remember the exact time of the manifestation of the disease. The first signs of Addison's disease include unexplained weakness, decreased performance, and decreased muscle strength. It becomes difficult for the patient to perform the usual household actions, in severe cases, the person does not leave the bed, refuses to eat and talk. Against the background of arterial hypotension, fainting occurs.
Most patients have dyspeptic disorders: diffuse abdominal pain, nausea and vomiting, unstable stool. Symptoms do not have a clear connection with the nature of nutrition, their intensity varies from mild malaise to serious disorders of digestive function. Due to massive losses of sodium, a predilection for salty food develops. Against the background of general weakness, decreased appetite and inhibition of anabolic processes, weight loss is observed.
In patients with Addison's disease, the skin darkens over time and acquires a characteristic bronze hue. This symptom caused the unofficial name of the pathology "bronze disease". First, hyperpigmentation occurs in open areas of the body that are exposed to sunlight, as well as in the area of the external genitals and nipples. Then the darkening of the skin is observed in places of constant friction with clothing, natural folds of the body.
The most dangerous consequence of Addison's disease is the development of acute adrenal insufficiency (Addison's crisis). This condition occurs against the background of trauma, surgery, severe infection. Patients develop hypotensive shock, severe dehydration, hypoglycemia within a few hours. In the absence of timely intensive therapy, an addisonic crisis leads to a fatal outcome.
More than 50% of patients eventually encounter mental disorders and neurosis-like conditions. This is especially typical for patients with an undiagnosed disease against the background of increased health anxiety, carcinophobia. With the progressive form of Addison's disease, hallucinations may occur. Hyperkalemia is fraught with the development of myocardiodystrophy, intraventricular blockades.
Examination of the patient by an endocrinologist begins with an assessment of clinical manifestations. To make a diagnosis, a complex of laboratory tests is necessarily carried out, the volume of which is determined taking into account the clinical situation, the general condition of the patient, the presence of signs of other autoimmune syndromes. Diagnosis of Addison's disease requires the following types of research:
With a detailed clinical picture of hypocorticism and the presence of bronze pigmentation, diagnosis is not difficult. In patients with erased manifestations of Addison's disease, it is necessary to carry out differential diagnosis with pernicious anemia, oncopathology, CNS diseases. Gastrointestinal symptoms should be distinguished from the clinic of "acute abdomen", chronic cholecystitis, enterocolitis.
Diet therapy is important for the correction of electrolyte disorders. The diet is enriched with table salt in an amount of 5-10 g per day. Since ascorbic acid activates the synthesis of corticosteroids, additional intake of vitamin C in high doses is recommended. To make up for the body weight deficit, the diet should contain a high level of protein, a sufficient amount of vegetable and animal fats in the right ratio.
If possible, etiotropic therapy is prescribed for Addison's disease. In case of tuberculous lesions of the adrenal glands, treatment is carried out under the supervision of a phthisiologist using an individual combination of drugs. For the control of autoimmune diseases, pathogenetic treatment is recommended: the appointment of levothyroxine, insulin therapy.
The basis of treatment is hormone replacement therapy, which involves the use of glucocorticoids and mineralocorticoids. Correction of mineralocorticoid metabolism is not particularly difficult, for this purpose, one drug is used in standard doses, which shows high efficiency. The use of corticosteroids has a number of rules and nuances:
The use of hormone therapy involves strict clinical and laboratory control to prevent the development of iatrogenic complications. The criteria for the success of therapy are the disappearance of subjective symptoms, gradual regression of pigmentation, maintenance of normal body weight. Patients with Addison's disease are advised to avoid taking diuretics that aggravate electrolyte disorders.
With properly selected replacement therapy, the disease does not significantly affect the quality and life expectancy of patients. The prognosis is determined by the patient's compliance, the causes and severity of manifestations of hypocorticism. Prevention of the disease consists in timely detection and treatment of tuberculosis patients, dynamic monitoring of patients with other autoimmune pathologies.