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Addison’s disease
What is it ?
Addison’s disease is characterized by the destruction of the two adrenal glands located above the kidneys, causing a deficit of the hormones they produce: cortisol and aldosterone. These hormones play a key role in functions such as regulating blood pressure and the body’s response to stress. Addison’s disease refers to any chronic primary adrenal insufficiency, as opposed to secondary adrenal insufficiency which originates in a deficiency of the hormone ACTH produced by the pituitary gland and in turn leads to a deficiency of cortisol. The cause of the disease is most often autoimmune but it can be more rarely infectious. It affects in the total population from 1 in 9 to 000 in 1 people in developed countries. (7)
Symptoms
They can occur at any age, but there is a peak around age 40 (1). They include: severe chronic fatigue, both physical and mental; gastrointestinal disorders (abdominal pain, diarrhea, vomiting, hypoglycemia, anorexia and weight loss); low blood pressure which can cause dizziness, lightheadedness and loss of consciousness; behavioral disturbances (concentration, irritability and depression).
Addison’s disease is sometimes referred to as “bronzing disease” because in some patients it causes brownish pigmentation of parts of the skin, especially in the folds, and of the mucous membranes. These hyperpigmentation spots can precede other symptoms by several years.
The symptoms of Addison’s disease vary from one individual to another, they evolve slowly over time and are not specific, which makes its diagnosis often late. It should be noted that, in rare cases, symptoms of the disease rapidly progress to acute adrenal insufficiency, constituting a medical emergency to prevent a fall in blood pressure and fatal kidney failure.
The origins of the disease
Between 75 and 80% of cases of Addison’s disease originate from an autoimmune disease: the immune system produces antibodies that gradually attack and destroy the adrenal cortex, corresponding to the outer envelope of the adrenal glands. (2)
In developed countries, only 10-15% of cases are caused by tuberculosis, whereas this infection was the main cause of Addison’s disease when it was described by the doctor of the same name in the middle of the XNUMXth century. . Other viruses and bacterial infections may be involved, such as Neisseria meningitidis (which causes meningitis) and the HIV virus. Other causes are even rarer: a tumor in the adrenal glands, their removal, a genetic anomaly causing a defect in the production of adrenal hormones …
Risk factors
The fact that the disease sometimes affects several members of the same family suggests that the disease occurs due to environmental factors (such as viral attack) in individuals with a genetic predisposition.
Prevention and treatment
In the past, this disease was serious and fatal and often caused by tuberculosis. The situation changed with the use of cortisone as an anti-inflammatory from the middle of the 20th century. Treatment for Addison’s disease consists of administering hormones replacement to those that the adrenal glands are no longer able to produce: corticosteroids tablets (hydrocortisone, prednisone, dexamethasone) in place of cortisol and a mineralocorticoid hormone (fludrocortisone) to replace aldosterone.
When hormone replacement therapy is well balanced and followed, the symptoms subside and the patient can lead a normal life and as long as the rest of the population. However, people affected by Addison’s disease are more exposed than others to the risk of a potentially fatal acute adrenal insufficiency crisis (or Addisonian crisis) which can occur following stress (infection, operation, accident, etc.). This is why people with Addison’s disease should carry a card with them indicating the disease, treatment and referring physicians.