Addison’s disease causes adrenal insufficiency linked to adrenal dysfunction, in which the adrenal glands do not produce enough hormones.
It involves a deficit in cortisol and aldosterone, hormones secreted by these glands and necessary for the proper functioning of the body.
The hormones aldosterone and cortisol, which are produced by the adrenal glands are essential for life, which makes this disease serious.
The adrenal glands are located above the kidneys and produce hormones useful for the functioning of various organs such as the liver, nervous system, cardiovascular system, etc. An adrenal gland is made up of two parts: the adrenal cortex (outer part) and the adrenal medulla (inner part). The adrenal cortex is responsible for the production of glucocorticoids (cortisol), mineralocorticoids (aldosterone) and androgens (testosterone). The adrenal medulla secretes catecholamines (adrenaline and norepinephrine).
In Addison’s disease, the patient suffers from a secretory insufficiency in glucocorticoid hormones, and sometimes in mineralocorticoid hormones.
The adrenal has two main areas, of which include:
- Outer cortex: produces steroid hormones (cortisol and aldosterone)
- Inner medulla: produces adrenaline
Addison’s disease is named after Thomas Addison, the British physician who first described the condition in the 1855 publication On the Constitutional and Local Effects of Disease of the Suprarenal Capsules. He originally described it as “suprarenal melasma”, but the scientific community later gave it the eponymous “Addison’s disease” in recognition of Addison’s discovery.
All six of Addison’s initial patients had adrenal tuberculosis, while the six from 1855 had adrenal tuberculosis; the term “Addison’s disease” does not imply an underlying disease process.
The condition was initially considered a form of anemia associated with the adrenal glands. Since little was known at the time about the adrenal glands (then called “supra-renal capsules”), Addison’s monograph describing the condition was an isolated insight. As adrenal function became better known, Addison’s study became known as an important scientific contribution and a classic example of careful medical observation.
Causes of Addison’s Disease
Addison’s disease is often caused by an autoimmune disease or tuberculosis.
It can thus be integrated into several autoimmune diseases:
- type 1 autoimmune polyendocrinopathy (genetic disease), rarely;
- type 2 autoimmune polyendocrinopathy, which is more common and is linked to the alteration of several genes which are not all known.
The latter disease most often associates thyroid damage (hypothyroidism or hyperthyroidism), type 1 diabetes and vitiligo.
There are also secondary causes of this kidney failure. This can be secondary to:
- bleeding under antioagulant;
- haemorrhages occurring during diseases which promote thrombosis;
- very rare infections.
Symptoms of Addison’s Disease
The symptoms of Addison’s disease can be very varied and set in insidiously. This explains why the diagnosis is often late and is only carried out in the presence of a serious complication, acute adrenal insufficiency, which requires emergency care.
The main symptoms of the disease are:
- loss of appetite;
- weight loss;
- a drop in blood pressure: in a person who had arterial hypertension, the blood pressure will require less and less treatment, or even disappear, the patient may become hypotensive.
Then, digestive disorders symptoms can be:
- abdominal pain;
The suggestive sign of Addison’s disease is melanoderma. It is a distorted pigmentation of the skin that worsens as the adrenals are destroyed. Initially, this symptom gives a nice color to the skin, but then the complexion appears earthy, grayish. Dark circles or scars may appear and the folds of the face appear more pronounced, black.
The sicker the patient, the more pronounced the pigmentation. This symptom sometimes sounds like Addison’s disease at first, but it can also delay diagnosis because doctors are more concerned about seeing a patient who looks bad than a tanned person.
Finally, signs of acute adrenal insufficiency occur with:
- severe dehydration;
- a significant drop in voltage;
In the blood, tests also show abnormalities:
- hyponatremia, which is a decrease in sodium levels;
- excess potassium;
- a tendency to hypoglycemia.
These signs appear quite late in the course of the disease.
Diagnostic of Addison’s Disease
After clinical evaluation, a diagnosis of Addison’s disease is commonly made using the ACTH test (also called the cosyntropin, tetracosactide, or Synacthen test), which is a specialized test that measures the amount of cortisol present in the blood and / or urine after a dose of a synthetic stimulating hormone Adrenocorticohormone is administered to the patient. Since Adrenocorticohormone stimulates the production of cortisol in the body, normal test results should show cortisol secretion. However, the results that are indicative of Addison’s disease will relate to the failure to test to cause the secretion of cortisol.
Prognosis (life expectancy)
People with Addison’s disease today have the same life expectancy as someone without.
Patients must take medication for the rest of their lives. If left untreated, Addison’s disease is always fatal, but if treated it has no effect on life expectancy . However, substitution treatment increases the risk of other diseases, including circulatory system.
The occurrence of adrenal crisis increases mortality (which can be prevented, for example, by educating the patient about the selection of the dose of drugs in the event of trauma and stress). If adrenal insufficiency was caused by neoplastic metastases, the prognosis is poor, and if tuberculosis is the cause, it depends on the extent of the infection .
Treatment of Addison’s Disease
Addison’s disease is treated with medications that serve to replace hormones that the body cannot produce. These drugs include hydrocortisone, which is used to raise cortisol, and the mineralocorticoid, which is used to raise aldosterone. Additionally, patients with Addison’s disease may also take doses of cortisol when experiencing acute illness or injury.
Once diagnosed and treated correctly, patients with Addison’s disease can lead normal lives. However, left untreated, cortisol levels can become dangerously low, which can eventually lead to adrenal crisis, which is a severe drop in blood sugar that leads to a cascade of other life-threatening complications.
Treatment of adrenal insufficiency is hormone replacement therapy based on cortisol or hydrocortisone (glucocorticoid) and fludrocortisone (mineralocorticoid):
- Hydrocortisone dose: The dose in adults is between 20 to 30 mg per day.
- Fludrocortisone dose: 0.05 to 0.1 mg orally daily, along with an increase in daily salt intake of 3 to 4 g.
Method of administration : the corticosteroid should be administered with meals, taking two thirds of the dose in the morning and one third in the late afternoon.
- Monitoring the effect of the medication: the levels of sodium, potassium, urea and creatinine in the blood must be known, together with the taking of blood pressure that should not be modified in the postural change, in addition to the improvement of all symptoms and control of body weight.
- Special considerations: all patients with chronic adrenal insufficiency must carry an identification card that alerts medical personnel to hospital admission or urgent medical attention, because in stressful situations, such as exercise, fever, surgical intervention, the amount must be doubled or tripled. dose of corticosteroids, and if it is not possible to administer it orally, it should be intravenously at a dose of 10 mg / h or 250-300 mg / day, to gradually decrease by 20-30% of the dose daily, when the stressful situation subsides. From hydrocortisone 100 mg / day, fludrocortisone does not need to be administered.
- Side effects: the main one is gastritis due to excess gastric acidity and disorders of the stomach lining, which is solved with antacids. If insomnia, irritability or mental excitement appear at the beginning of treatment, the dose should be reduced.
Information: Cleverly Smart is not a substitute for a doctor. Always consult a doctor to treat your health condition.
Photo description: Acanthosis nigricans (a manifestation of the skin characterized by hyperpigmented areas, poorly demarcated, which typically appear in skin folds level, such as: neck, navel, groin, armpits. The skin is thickened and velvety, darker in color (from brownish to black) than the surrounding areas) in a person with Addison’s disease.