Synonyms: Addison's disease, "bronze disease", chronic adrenal insufficiency, adrenal insufficiency.
Scientific editor: Volkov AA, endocrinologist, practical experience since 2015.
September, 2018.
Addison's disease - endocrine pathology associated with insufficient production of adrenal hormones vitally necessary. It is a consequence of failure in one of the working units of the hypothalamic-pituitary-adrenal system.
Addison's disease occurs when lesions of more than 90% of adrenal tissue. Pathology rough estimates found in 1 patient out of 20 thousand. In most cases the cause of the disease - an autoimmune process (attack its own immune system), followed in frequency to be tuberculosis.
As syndrome, chronic adrenocortical insufficiency is present at a plurality of inherited diseases.
Causes
- autoimmune lesion of the adrenal cortex (the immune system attacks its own)
- adrenal tuberculosis
- removal of the adrenal glands
- the effects of long-term hormone therapy
- fungal diseases (histoplasmosis, blastomycosis, coccidioidomycosis)
- sarcoidosis
- bleeding in the adrenal glands
- tumor
- amyloidosis
- Acquired immunodeficiency syndrome (AIDS)
- syphilis
- variations in the genetic code
- adrenoleukodystrophy.
Symptoms of Addison's disease
Addison's disease is accompanied by increased levels of adrenocorticotropic hormone (ACTH) Together with alpha-melanocyte stimulating hormone, causes skin darkening and mucous membranes - the hallmark of Addison's disease, and therefore it is also called "Bronze".
Secondary insufficiency of the adrenal cortex caused by iron deficiency of the brain - the pituitary gland; Unlike primary never accompanied by a darkening of the skin.
Manifestation of Addison's disease are made up of signs of inadequate allocation of adrenal hormones. The predominance of certain manifestations determined by the duration of the disease.
- Darkening of the skin and mucous membranes (when primary failure) often for months or years ahead of other manifestations. Darkening of the skin due to the constant stimulation kortikotrofov. The simultaneous presence of vitiligo (Lightening skin in chaotic areas) due to autoimmune destruction of melanocytes, the cells responsible for skin coloration.
- Extreme weakness (especially muscle), fatigue, weight loss, poor appetite.
- lowering blood pressure (hypotension), Which is accompanied by vertigo. Also due to low blood pressure, patients reported poor cold tolerance.
- Signs of the gastrointestinal tract: nausea, vomiting, occasional diarrhea.
- There are mental disorders (depression, psychosis).
- Strengthening of taste, olfactory, auditory sensitivity; may cause an irresistible desire to salty foods.
Adrenal (adrenal) crises
Adrenal crisis - urgently develop a condition that threatens the health and life of the patient, accompanied by a sharp decline levels of adrenal hormones in the bloodstream, or a sudden increase in their needs, provided that the function of organs broken.
Causes of adrenal crisis:
- Stress: acute infectious disease, trauma, surgery, emotional stress and other stress factors. Adrenal crises in these situations provoke a lack of adequate increasing doses of hormone replacement therapy.
- Bilateral adrenal hemorrhage.
- Bilateral adrenal artery embolism or thrombosis adrenal veins (e.g., during indwelling Research).
- Adrenalectomy without adequate replacement therapy.
Manifestations of adrenal crisis:
- lowering blood pressure,
- stomach ache,
- vomiting
- impaired consciousness.
Diagnostics
Diagnosis is an endocrinologist and is reduced to the identification of lack of functionality adrenocortical capacity (increase synthesis of the hormone cortisol in response to stimulating influence).
Primarily recommended cortisol assessment in the morning. If serum cortisol index at 8.00 in the morning less than 3 .mu.g / dL indicate adrenal insufficiency.
In the biochemical analysis of blood can be detected hyponatraemia and hypokalemia. If there is a predisposition to the disease of Addison is recommended annually monitor the ACTH levels in the blood. There is a gradual increase in hormone level to the upper limit of normal (50 pg / ml).
Treatment of Addison's disease
Therapy
Adrenal hormone replacement therapy. Apply hydrocortisone and fludrocortisone.
- Hydrocortisone 10 mg in the morning and 5 mg orally every day after dinner (adults up to 20-30 mg / day.). Alternative hydrocortisone - prednisolone received 1 per day.
- Fludrocortisone at 0.1-0.2 mg orally 1 time a day.
the dose should be reduced by increasing blood pressure. In acute illness (e.g. common cold), or after minor trauma double dose hormones up to feel better.
In the surgical treatment before and (if necessary) after correcting operations dose hormones. Liver diseases and elderly patients doses of drugs should be reduced.
Woman putting appointment androgen therapy. Men are such addition is not necessary, since they are produced in sufficient quantities androgens in the testes.
Diet in Addison's disease:
- A sufficient amount of proteins, fat, carbohydrates, and vitamins, especially B and C (recommended hips broth, black currants, brewer's yeast).
- Table salt consumed in large amounts (20 g / d).
- The diet reduced content of potato, peas, beans, legumes, dried fruits, coffee, cocoa, chocolate, nuts, mushrooms.
- Vegetables, meat, fish should be consumed cooked form.
- Diet fractional before bedtime is recommended a light dinner (glass of milk).
sources:
- G.A.Melnichenko, E.A.Troshina, M.Yu.Yukina, N.M.Platonova, D.G.Beltsevich. clinical recommendations Russian association of endocrinologists on the diagnosis and treatment of primary adrenal insufficiency in adults (draft). - Consilium Medicum. 2017; 4: 8-19
