Addison's disease

Description:

Primary hypocorticoidism of an autoimmune and tubercular etiology is designated as Addison's disease.

Symptoms of the Disease of Addison:

The main clinical symptoms of primary hypocorticoidism (Addison's disease) are:

  1. Hyperpegmentation of skin and mucous. Expressiveness of a hyperpegmentation corresponds to weight and prescription of process. In the beginning open parts of a body, the most subject to action of the sun, - a face, a neck, hands, and also places which also normal are stronger pigmented (nipples, a scrotum, external genitals) darken. The hyperpegmentation of places of friction and visible mucous membranes has great diagnostic value. Are characteristic a hyperpegmentation of palmar lines which are allocated on rather light background of surrounding fabric, darkening of places of friction with clothes, a collar, a belt. The shade of skin can vary from smoky, bronze (bronzed disease), colors of suntan, dirty skin, up to the expressed diffusion hyperpegmentation. It is rather specific, but the hyperpegmentation of mucous membranes of lips, gums, cheeks, a soft and hard palate is not always expressed. Lack of a melanoderma is rather serious argument against the diagnosis of primary chronic adrenal insufficiency, nevertheless cases of a so-called "white addisonizm" when the hyperpegmentation is almost absent meet. Against the background of a hyperpegmentation pigment-free spots (vitiligo) often are found in patients. Their size fluctuates from small, scarcely noticeable, to large, with the wrong contours, brightly allocated on the general dark background. Vitiligo is characteristic only for primary chronic adrenal insufficiency of autoimmune genesis.
  2. Weight loss. Its expressiveness fluctuates from moderated (3-6 kg) to considerable (15-25 kg), especially at initially excess body weight.
  3. General weakness, adynamy, depression, decrease in a libido. The general weakness progresses from easy, before total loss of working capacity. Patients are suppressed, sluggish, are not emotional, irritable, depressive frustration are diagnosed for a half of them.
  4. Arterial hypotension can have only ortostatiyesky character in the beginning; patients often report about unconscious states which are provoked by various stresses. Detection at the patient of arterial hypertension - a weighty argument against the diagnosis of adrenal insufficiency though if primary chronic adrenal insufficiency developed against the background of the previous idiopathic hypertensia, arterial pressure can be normal.
  5. Dispepsichesky frustration. The small appetite and diffuse pains in epigastriums, alternation of ponos and locks are most often noted. At the expressed decompensation of a disease nausea, vomiting, anorexia develop.
  6. Addiction to salty food is connected with the progressing sodium loss. In some cases it reaches the salt use in pure form.
  7. The hypoglycemia in the form of typical attacks practically does not meet, but can come to light at a laboratory research.

The clinical picture of acute adrenal insufficiency (addisonichesky crisis) is presented by a complex of symptoms on which dominance it is possible to allocate three clinical forms:

  1. Cardiovascular form. At this option the phenomena of an acute circulatory unefficiency dominate: pallor of the person with a Crocq's disease, a cold snap of extremities, the expressed arterial hypotonia, tachycardia, threadlike pulse, an anury, a collapse.
  2. Gastrointestinal form. On symptomatology can remind food toxicoinfection or even a condition of an acute abdomen. Abdominal pains of spastic character, nausea, pernicious vomiting, a liquid chair, a meteorism prevail.
  3. Psychological form. The headache, meningeal symptoms, spasms, focal symptomatology, nonsense, block, a stupor prevail.

Etiologies of Addison:

Are the main reasons for primary chronic adrenal insufficiency:

  1. Autoimmune destruction of bark of adrenal glands (98% of cases). In blood of most of patients antibodies to enzyme 21 to a hydroxylase (R450s21) are found. Besides, at 60-70% of patients other autoimmune diseases, most often, an autoimmune thyroiditis develop.
  2. Adrenal tuberculosis (1-2%). Most of patients suffers from tubercular damage of lungs.
  3. Adrenoleykodistrofiya (1-2%) - the H-linked recessive disease at which defect of the fermental systems which are carrying out exchange of long-chain fatty acids is inherited results from them the accumulation in the TsNS white matter and bark of adrenal glands leading to dystrophic changes.
  4. The rare reasons (coagulopathies, a dustoronny hemorrhagic heart attack of adrenal glands (Waterhouse-Frideriksen's syndrome) metastasises of tumors, a bilateral adrenalectomy, the HIV-associated complex. Various tumoral and destructive processes in gipotalamo-pituitary area are the main reasons for a secondary hypocorticoidism. Acute adrenal insufficiency (addisonichesky crisis), in most cases, has the same reasons, as chronic and represents its decompensation.

Treatment of the Disease of Addison:

Includes lifelong replacement therapy by drugs of corticosteroids. After diagnosis in most cases treatment begins with intramuscular or intravenous administration of a hydrocortisone (100-150 mg/days on 2-3 injections). The expressed positive effect from therapy by a hydrocortisone is the important testimony of correctness of the established diagnosis.

After stabilization of a condition of the patient from chronic adrenal insufficiency the maintenance therapy is appointed: a combination of drugs with glyuko-and mineralokortikoidny activity.

Treatment of acute adrenal insufficiency is based on the following basic principles:

    * Rehydration therapy: isotonic solution in volume of 2-3 liters for the first time days in combination with 10-20% glucose solution.
    * Replacement therapy by a hydrocortisone: 100 mg in/in, then each 3-4 hours on 50-100 mg in/in or in oil. In process of stabilization of a condition of the patient the dose decreases to supporting. In the absence of a hydrocortisone, for example for the period of delivery of the patient in clinic purpose of equivalent doses of Prednisolonum is possible.
    * Symptomatic therapy of the associated diseases which caused a decompensation of chronic adrenal insufficiency (most often - antibacterial therapy of infectious diseases).