People with Addison's disease require hormone replacement therapy throughout their lives. This condition can appear at any age. Given the disability caused by the symptoms of this disease, it is important to recognize them as early as possible in order to set up a treatment that will limit the aftereffects.

1. Addison's disease: definition

According to the portal of rare diseases and orphan drugs in its article dedicated to Addison's disease, this pathology affects between 1 and 5 people out of 10 000 in France. It owes its name to the first doctor to describe the symptoms of this disease, Thomas Addison, as indicated by the Adrenal Association, which brings together people suffering from pathologies affecting the glands located above the kidneys.

When a person has Addison's disease, the adrenal glands do not produce enough cortisol and aldosterone. These hormones are necessary for the body's metabolism:

Cortisol allows the body to have an adequate response to any kind of stress. This stress can be caused by a professional or personal situation. It can also be caused by the announcement of an illness, trauma, or by the anticipation of a surgical operation. Cortisol plays a role in regulating blood pressure, heart and immune system function, and blood sugar levels;
Aldosterone is a hormone that regulates sodium and potassium levels in the blood. It also controls the amount of fluid excreted by the kidneys in the form of urine, which has an impact on blood volume and blood pressure.

In its article on the disease, the French Society of Endocrinology states that Addison's disease is referred to by physicians as "primary adrenal insufficiency". And that it should not be confused with a related disease, "high adrenal insufficiency": this occurs when the pituitary gland, a gland located at the base of the brain, does not secrete enough adrenocorticotropic hormone (ACTH), which is necessary for the adrenal glands to secrete cortisol. These two conditions do not have the same cause or consequences and therefore do not require the same treatment.

2. The symptoms of Addison's disease

The destruction of the adrenal glands usually occurs slowly. The symptoms of Addison's disease appear over the years. The most common are:

Abnormal menstrual periods in women;
a desire to eat salty foods associated with a very low blood sugar level;
gastroenterological disorders: loss of appetite or anorexia, diarrhea, nausea, vomiting, abdominal pain
mood disorders: depression, irritability, psychological exhaustion
asthenia (severe fatigue), accompanied by dizziness or vertigo, muscle weakness
low blood pressure;
hyperpigmentation of the skin, especially around scars, skin folds, and joints;
Unexplained weight loss.

However, symptoms can also appear suddenly: doctors refer to this as acute adrenal insufficiency, when the person is faced with an injury, illness, burn, surgery, or a period of intense stress. In these cases, the most obvious symptoms are:

Sudden weakness ;
Severe pain in the abdomen, lumbar region, or legs
A total loss of consciousness;
A high fever or, on the contrary, a drastic decrease in body temperature.

If not treated immediately by emergency medical services, the person suffering from acute adrenal insufficiency may die.

3. The causes of Addison's disease

Addison's disease is an autoimmune disease. That is, the person suffering from this adrenal insufficiency has his or her immune system (which is supposed to protect him or her from infections) attacking his or her own organs and tissues: in this case, the outer part of the adrenal glands, where cortisol and aldosterone are produced. According to the French Society of Endocrinology in its article on Addison's disease, the autoimmune cause of this pathology would explain about 8 cases out of 10.

Other causes of Addison's disease are :

tuberculosis, which is involved in about 20% of cases, according to the French Society of Endocrinology;
human immunodeficiency virus (HIV) infection, which causes AIDS;
Adrenoleukodystrophy, an X-linked disease - hence the higher prevalence in women than in men;
Congenital adrenal hyperplasia, which can be detected in utero;
iatrogenic causes, i.e. following surgery (removal of both adrenal glands) or medication
cancer, in particular, lung, kidney, breast, or ENT cancer;
Lymphoma;
sarcoidosis, an inflammatory disease that causes clusters of cells to form in the organs;
amyloidosis, which causes abnormal accumulation of certain proteins in the organs.

4. Addison's disease: when to consult?

It is advisable to consult your doctor if you have symptoms that are common in Addison's disease, such as hyperpigmentation, intense, and chronic fatigue, unexplained weight loss, gastrointestinal problems (nausea, vomiting, and abdominal pain) with no immediate cause, dizziness or lightheadedness, an irrepressible need to eat very salty foods, muscle or joint pain.

In addition, when faced with symptoms of acute adrenal insufficiency, it is necessary to go urgently to your doctor or to the hospital. As a reminder, the clinical signs of acute adrenal insufficiency are:

very severe asthenia ;
intense pain in the abdomen, lumbar region, or legs
malaise;
High fever or a sudden drop in body temperature.

5. Testing and diagnosis of Addison's disease

To diagnose Addison's disease, your doctor will ask you about the symptoms and then perform a clinical examination. If your doctor sees dark spots on your skin, he or she may suspect Addison's disease and order additional tests, including:

blood tests: these measure blood sodium, potassium, cortisol, and plasma adrenocorticotropic hormone (ACTH) levels;
An ACTH stimulation test, to assess the response of the adrenal glands after the injection of synthetic ACTH. If the adrenal glands secrete a low amount of cortisol after this administration, it is a sign that they are dysfunctional;
a chest x-ray to look for calcium deposits on the adrenal glands;
Computed tomography (CT scan of the adrenals): with this technique, which combines numerous cross-sectional X-ray images, the doctor can evaluate the adrenal glands and/or pituitary gland.

6. Treatments for Addison's disease

The treatment of Addison's disease is mainly based on the use of hormone replacement therapy, which is necessary for the entire life of the patient. Indeed, this pathology cannot be cured, and it is necessary to compensate for the levels of hormones that the body does not produce.

The French Society of Endocrinology, in its file on adrenal insufficiency, indicates the first-line treatments:

hydrocortisone, which replaces cortisol;
fludrocortisone acetate as a substitute for aldosterone.

In addition, it is advisable to modify your diet to consume more salt (sodium), especially if you have to exert yourself, when it is hot, or if you suffer from gastrointestinal problems. It may be necessary to temporarily increase the dosage of your treatment when under stress. However, never do so without first consulting your doctor. This increase in hormone replacement may be necessary if your body is under severe stress, such as after surgery, infection, or injury. Some people with Addison's disease worry about the side effects of their hormone replacement therapy. However, side effects are not expected to occur, as the ingested dose simply replaces the missing amount. There is no risk of overdosing.

The Adrenal Association, in its article on Addison's disease, recommends that you:

Carry a card indicating that you have Addison's disease at all times so that if you become unwell, emergency services will know immediately what treatment to give you. The card can also tell you what medication to give and how much to give you if you have acute adrenal insufficiency;

Always carry your medication with you, and do not interrupt your treatment for any reason;

Have an annual check-up with your doctor or endocrinologist.

7. How can Addison's disease be prevented?

Addison's disease cannot be prevented. However, once the diagnosis has been made, there are steps you can take to prevent acute adrenal failure:

See your doctor as soon as you feel unusually tired, fatigued, or if you lose a lot of weight without dieting;
Ask your doctor how much you can increase the amount of hormones you take if you are under intense stress;
Go to the emergency room if you feel sick or vomit.

Sources :

Le Manuel MSD (version professionnelle), Société française d’endocrinologie, le portail des maladies rares et des médicaments orphelins, Association Surrénales