Addison's disease is a rare disease. It is an adrenal insufficiency: the body no longer produces cortisol and aldosterone, two hormones secreted by the adrenal glands.
Addison's disease is an adrenal insufficiency due to the destruction of the adrenal cortex glands.
It is a deficiency of cortisol and aldosterone, hormones secreted by these glands and necessary for the proper functioning of the body.
Addison's disease is a particular cause of adrenal disease.
is a very rare condition.
Addison's disease is usually caused by an autoimmune disease or tuberculosis.
Therefore, it can be integrated into several autoimmune diseases:
- autoimmune polyhedrinopathy type 1 (genetic disease), rarely
- autoimmune polyhedrinopathy type 2, which is more frequent and linked to the alteration of several genes, not all of which are known.
The latter disease is most often associated with thyroid damage (hypothyroidism or hyperthyroidism), type 1 diabetes and vitiligo.
There are also secondary causes of kidney failure. It can be secondary to..:
- bleeding with anti-coagulant medication;
- hemorrhages from diseases that promote thrombosis;
- very rare infections.
The symptoms of Addison's disease can be very varied and can develop insidiously.
This explains why the diagnosis is usually late and is only made in the face of a serious complication, acute adrenal insufficiency, which requires treatment in an emergency room.
The main symptoms of the disease are:
- loss of appetite;
- weight loss ;
- a decrease in blood pressure: in a person with high blood pressure, the pressure will require less and less treatment, or even disappear, as the patient may become hypotensive.
Then, digestive problems can appear:
- abdominal pain;
The telltale sign of Addison's disease is melanodermia. It is a distorted pigmentation of the skin that worsens as the adrenals are destroyed.
Initially, this symptom gives the skin a nice color, but then the complexion appears earthy, grayish. Dark circles or scars may appear and the folds of the face appear more marked, black.
The sicker the patient, the more pronounced the pigmentation.
This symptom sometimes reminds us of Addison's disease, but it can also delay the diagnosis because doctors are more concerned about a patient who looks bad than a person who is tanned.
Finally, signs of acute adrenal failure occur with:
- severe dehydration;
- a significant voltage drop;
- a fever.
In the blood, the tests also show abnormalities:
- hyponatremia, a decrease in sodium levels;
- an excess of potassium;
- a tendency to hypoglycemia.
These signs appear quite late in the course of the disease.
The diagnosis of adrenal insufficiency is based on the test of synactin, a drug that stimulates the production of cortisol by the adrenal glands.
It consists of injecting 250 micrograms of synactin intravenously or intramuscularly and measuring the production of cortisol at the end of the test.
Some doctors do it in thirty minutes, others in sixty minutes.
When the cortisol level is below a certain threshold (it depends on each laboratory, but it is usually around 200 nanograms per milliliter), the doctor can say that it is indeed adrenal insufficiency.
The doctor then checks for Addison's disease and doses ACTH, a hormone secreted by the pituitary gland, which should stimulate cortisol and aldosterone production if the adrenals are working properly, as well as renin (an enzyme of the renin-angiotensin-aldosterone system).
During this test, the affected person is injected with ACTH and has a blood test.
If the two hormones are not detected or if the abnormal presence of ACTH is found in the blood, it means that the pituitary gland is trying to stimulate the adrenal cortex glands without success.
The renin assay is used to determine if circulating aldosterone or renin levels are abnormal.
Treatment of Addison's disease is based on replacement of the deficient hormones cortisol and aldosterone.
In France, cortisol is replaced by hydrocortisone, a product usually prescribed in a dose of 15 to 25 milligrams per day in two or three doses. This treatment is absolutely essential and should not be stopped.
Aldosterone is replaced by fludrocortisone, available in two forms in France:
- Fludrocortisone 10 micrograms, mainly for children and only available in hospital pharmacies;
- Fludrocortisone 50 micrograms, available from November 2012 under the name Flucortac in the city pharmacies. This drug is strictly identical to Adixone, the name by which it was previously distributed. The production of Adixone had to be discontinued in April 2012 following the shutdown of two Arkopharm facilities manufacturing it.
The treatment is then adapted by the physician according to the patient's clinical condition and hormone levels.
It is followed for life without interruption, since interrupting treatment for a few days can have dramatic consequences for the patient's health.
Patient education is essential
The physician must teach the patient how to adapt his treatment.
For example, when a person develops an acute illness (gastroenteritis, infection...), practices intense sports activities or falls prey to very strong emotions, the adrenal cortical glands - when they are functioning - produce two or three times more cortisol.
In these cases, the patient must double or even triple his dose of hydrocortisol by mouth or injection.
This learning is fundamental. If it is not acquired, the patient runs the risk of an acute adrenal crisis, a great therapeutic urgency that compromises the vital prognosis.
During seizures, the patient should carry an adrenal insufficiency card at all times. In addition, you should receive an injection of hydrocortisone, hydrocortisone and fludrocortisone tablets in advance so that you are not left out of treatment.
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