Chronic adrenal insufficiency

Adrenal insufficiency (synonym: hypocorticism) - a decrease in the glucocorticoid and mineralocorticoid function of the adrenal glands - one of the most severe diseases of the endocrine system.

Acute and chronic adrenal insufficiency are distinguished. Primary hypocorticism, caused by direct damage to the adrenal cortex, and secondary hypocorticism, associated with a pituitary or hypothalamic defect, accompanied by ACTH deficiency, are distinguished.

ICD-10 code

• E27.1 Primary adrenal cortex insufficiency.
• E27.3 Drug-induced adrenal cortex insufficiency.
• E27.4 Other and unspecified adrenal cortex insufficiency.

Causes of Chronic Adrenal Insufficiency

The main cause of primary hypocorticism is autoimmune destruction of the adrenal cortex. The appearance of autoantibodies to adrenal cell antigens is often accompanied by the production of other organ-specific autoantibodies, which leads to the development of polyendocrine syndrome - a combination of chronic adrenal insufficiency with autoimmune thyroiditis, diabetes mellitus, hypoparathyroidism, pernicious anemia, vitiligo, and candidiasis. Another cause of primary adrenal damage is tuberculosis, although tuberculous etiology is less common in children than in adults. Sometimes primary hypocorticism can be caused by congenital hypoplasia of the adrenal cortex, a genetically determined disease with a recessive type of inheritance linked to the X chromosome (occurs only in boys).

The most common cause of secondary hypocorticism is destructive processes in the hypothalamic-pituitary system (tumor, trauma, infection).

Due to the deficiency of glucocorticoids, which activate gluconeogenesis in a healthy organism, glycogen reserves in muscles and liver decrease, and glucose levels in blood and tissues decrease. Reduced glucose supply to tissues leads to adynamia and muscle weakness. Deficiency of mineralocorticoids causes increased excretion of sodium, chlorides and water, which leads to hyponatremia, hyperkalemia, dehydration and decreased blood pressure. Deficiency of adrenal androgens, accompanying adrenal insufficiency, is manifested by delayed growth and sexual development. At the same time, the intensity of anabolic processes in bone and muscle tissues decreases. Clinical signs of chronic adrenal insufficiency appear with the destruction of 90% of glandular cells.

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Symptoms of Chronic Adrenal Insufficiency

Symptoms of chronic adrenal insufficiency are primarily caused by glucocorticoid deficiency. Congenital forms of hypocorticism manifest themselves in the first months of life. In autoimmune adrenalitis, the onset of the disease is more often after 6-7 years. Characteristic are lack of appetite, weight loss, decreased blood pressure, asthenia. Abdominal pain, nausea, and causeless vomiting are often noted.

Hyperpigmentation of the skin is a pathognomonic clinical symptom of primary hypocorticism. Natural folds of the skin and areas of contact with clothing are intensely colored. Hyperpigmentation is caused by excessive secretion of ACTH and melanocyte-stimulating hormone. In secondary hypocorticism, hyperpigmentation is absent.

In some cases, hypoglycemic conditions develop due to the absence of counter-insular action of glucocorticoids.

In congenital chronic adrenal insufficiency, signs of the disease appear soon after birth. Rapid weight loss, hypoglycemia, anorexia, and regurgitation are characteristic. Pigmentation of the nipples, white line of the abdomen, and external genitalia against a background of pale skin are typical.

Classification of chronic adrenal insufficiency

I. Primary adrenal insufficiency (Addison's disease)

• Congenital.
  • Congenital hypoplasia of the adrenal cortex.
  • Hypoaldosteronism.
  • Adrenoleukodystrophy.
  • Familial isolated glucocorticoid deficiency.
  • Allgrove's syndrome.
• Acquired.
  • Autoimmune adrenalitis.
  • Infectious adrenalitis (tuberculosis, syphilis, mycosis).
  • Amyloidosis.
  • Metastases of malignant tumors.

II. Secondary adrenal cortex insufficiency

• Congenital.
  • Isolated corticotropin deficiency.
  • Hypopituitarism.
• Acquired.
• Destructive damage to the pituitary gland (tumors, hemorrhage, infections, autoimmune hypophysitis).

III. Tertiary adrenal cortex insufficiency

• Congenital.
• Isolated corticoliberin deficiency.
• Multiple hypothalamic insufficiency.
• Acquired.
• Destructive lesion of the hypothalamus.

IV. Impaired reception of steroid hormones

• Pseudohypoaldosteronism.
• Iatrogenic adrenal cortex insufficiency.

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Complications of chronic adrenal insufficiency

Without treatment, the symptoms of chronic adrenal insufficiency rapidly increase, and an adrenal insufficiency crisis develops, characterized by symptoms of acute adrenal insufficiency. There is severe weakness, a drop in blood pressure, vomiting, loose stools, abdominal pain. Clonic-tonic seizures and meningeal symptoms are possible. Symptoms of dehydration and cardiovascular insufficiency increase. If treatment is untimely or inadequate, a fatal outcome is possible.

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Diagnosis of chronic adrenal insufficiency

The main diagnostic criterion for hypocorticism is a decrease in the content of cortisol and aldosterone in the blood serum. In primary hypocorticism, low levels of cortisol and aldosterone are accompanied by an increase in the content of ACTH and renin in the blood plasma.

Glucocorticoid deficiency results in hypoglycemia. Mineralocorticoid deficiency is characterized by hyperkalemia and hyponatremia.

In latent forms of chronic adrenal insufficiency, a stimulation test with ACTH is performed: after blood sampling to determine the basal level of cortisol, a prolonged-release ACTH drug, tetracosactide, or a fast-acting drug, syncorpine, or corticotropin, is administered at 8 a.m. Then the cortisol level is determined again 12-24 hours after the administration of tetracosactide or 60 minutes after corticotropin. In healthy children, the cortisol content after the administration of ACTH is 4-6 times higher than the basal level. The absence of a response to stimulation indicates a decrease in the reserves of the adrenal cortex.

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Differential diagnostics

Differential diagnosis of chronic adrenal insufficiency should be carried out with neurocirculatory dystonia of the hypotonic type, essential arterial hypotension. The combination of arterial hypotension with weight loss can be in gastric ulcer, nervous anorexia, oncological pathology. In the presence of hyperpigmentation, differential diagnosis is carried out with dermatomyositis, scleroderma, pigment-papillary dystrophy of the skin, poisoning with salts of heavy metals.

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Treatment of chronic adrenal insufficiency

Treatment of the crisis is aimed at eliminating electrolyte disturbances and hypoglycemia. Infusion therapy includes 0.9% sodium chloride solution and 5% glucose solution. The total volume of fluid is calculated based on physiological needs, taking into account losses.

At the same time, replacement therapy is started - preference is given to glucocorticosteroids with mineralocorticoid activity, for example, hydrocortisone. The drug is administered fractionally throughout the day intramuscularly; in severe cases, water-soluble hydrocortisone preparations are administered intravenously. Subsequently, after the crisis of adrenal insufficiency has been relieved, patients are transferred to permanent replacement therapy with tablets (cortef, cortineff). The adequacy of treatment is assessed by the parameters of physical and sexual development, blood pressure, the level of electrolytes in the blood plasma, and ECG.

Forecast

With adequate replacement therapy, the prognosis for life is favorable. In intercurrent diseases, injuries, stressful situations, there is a risk of developing a crisis of adrenal insufficiency. In cases of suspected risk, the daily dose of glucocorticosteroids should be increased by 3-5 times. During surgical intervention, the drugs are administered parenterally.

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