What is it ?
Addison’s disease is characterized by the destruction of the two adrenal glands located above the kidneys, causing a deficiency of the hormones they produce: cortisol and aldosterone. These hormones play a key role in functions such as the regulation of blood pressure or the body’s response to stress. Addison’s disease refers to any chronic primary adrenal insufficiency, as opposed to secondary adrenal insufficiency, which is caused by a deficiency of the pituitary hormone ACTH and in turn causes cortisol deficiency. The cause of the disease is most often autoimmune but it can be infrequently infectious. It affects the total population of 1 in 9,000 to 1 in 7,000 people in developed countries.
Symptoms of Addison’s disease
They can occur at any age, but there is a peak around 40 years. They include: chronic fatigue, physical and mental; gastrointestinal disorders (abdominal pain, diarrhea, vomiting, and hypoglycemia, anorexia and weight loss); have low blood pressure that may cause dizziness, lightheadedness, and unconsciousness behavioral problems (concentration, irritability and depression).
Addison’s disease is sometimes referred to as the “tan disease” because it causes, in some patients, a brownish pigmentation of parts of the skin, especially in the folds, and mucous membranes. These hyperpigmentation tasks may 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 his diagnosis often late. It should be noted that, in rare cases, the symptoms of the disease progress rapidly to acute adrenal insufficiency, constituting a medical emergency to prevent a fatal fall in blood pressure and kidney failure.
The origins of the disease
Between 75 and 80% of cases of Addison’s disease are caused by an autoimmune disease: the immune system produces antibodies that attack and progressively destroy the adrenal cortex, corresponding to the outer envelope of the adrenal glands.
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 19th century. . Other viruses and bacterial infections may be involved, such as Neisseria meningitidis(which causes meningitis) and the HIV virus . Other causes are even more rare: a tumor in the adrenal glands, their removal, and a genetic anomaly causing a defect in the production of adrenal hormones …
Risk factors for Addison’s disease
The fact that the disease sometimes affects several members of the same family suggests that the disease is due to environmental factors (such as viral attack) in individuals with a genetic predisposition.
Prevention and treatment of Addison’s disease
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 drug from the middle of the 20th century. The treatment of Addison’s disease consists in administering hormone replacement to that which the adrenal glands are no longer able to produce: corticosteroid tablets (hydrocortisone, prednisone, and dexamethasone) to replace cortisol and a mineralocorticoid hormone (fludrocortisone) to substitute for aldosterone.
When hormone replacement therapy is well balanced and followed, the symptoms regress and the patient can lead a normal life as long as the rest of the population. However, people with Addison’s disease are more exposed than others to the risk of a crisis of acute adrenal insufficiency potentially fatal (or Addisonian crisis) that can occur as a result of stress (infection, operation, accident …). For this reason, people with Addison’s disease should carry a card with the disease, treatment and referring doctors.