Addison’s disease – primary and secondary, causes, diagnosis, principles of treatment

The disease owes its name to the name of Tomasz Addison, the surgeon who first described its symptoms and causes. It is also sometimes called chisawica. This is chronic adrenal insufficiency caused by insufficient production of hormones by the adrenal cortex.

Such a condition may be a consequence of tuberculosis, cancer or the process of autoimmunity, i.e. self-destruction of the organism. The disease usually leads to serious disturbances in the water, electrolyte and carbohydrate balance.

Medical statistics show that Addison’s disease affects 4-11 people out of 100. It can develop at any age, but the highest incidence is recorded in the 3rd and 4th decade of life. Both men and women are at risk, but women get sick much more often than men.

A bit of anatomy

A healthy person has two adrenal glands that are located above each kidney. The adrenal glands are the endocrine glands. Their inner core, the core, produces epinephrine, also known as adrenaline, which the body produces when stressed. It is adrenaline that allows us to react quickly in the moment of danger. Its effect on the body manifests itself in accelerating the pulse, increasing blood pressure and increasing blood glucose levels.

The outer part of the adrenal gland, called the cortex, produces two steroid hormones that are very important for the proper functioning of the body – cortisol and aldosterone. Cortisol has many functions – it regulates the metabolism of proteins, carbohydrates and fats, determines the body’s response to inflammation, stimulates the liver to increase blood glucose levels, and helps to control the amount of water in the body. Aldosterone regulates salt and water levels, which determine blood pressure.

Primary and secondary disease

The production of cortisol is regulated by the adrenocorticotropic hormone (ACTH), which is produced in the pituitary gland. The classic form of Addison’s disease is the impairment of cortisol and aldosterone secretion as a result of severe damage to both adrenal glands. This clinical condition is called primary adrenal insufficiency. It also happens otherwise. ACTH deficiency can lead to decreased cortisol production, yet aldosterone levels will remain within the normal range. Then we are dealing with secondary adrenal insufficiency, which specialists describe as a condition similar to Addison’s disease, because the body does not release cortisol.

Where to look for the reasons?

Before antibiotics came into widespread use, complications from tuberculosis were the main cause of the disease. It is currently considered that over 50 percent. cases of the disease are the result of inappropriate autoimmune processes, i.e. those when the body’s immune system produces antibodies against the cells of the adrenal cortex and slowly destroys them. The self-destruction process can take months or even years. Other causes of Addison’s disease include chronic infections, certain types of mycoses, AIDS-related cytomegalovirus (CMV) infection, and tumor metastasis (often in breast cancer). The disease can also develop after the surgical removal of both adrenal glands.

symptoms

The slow decline in cortisol and aldosterone levels in the body leads to increased fatigue, loss of appetite and weight loss. Blood pressure is low, which is painfully felt by patients with dizziness appearing while standing. There are also other unpleasant symptoms of the developing disease. These include nausea, sometimes with vomiting and diarrhea. The muscles are weakened and the sick often suffer from cramps. Mood changes also appear, often irritating or even depressed. Salt deficiency in the body manifests itself in an unbridled craving for salty food. A sign of the disease may also be a tan, or rather a change in the color of the skin or mucous membranes on the gums.

Most people with Addison’s disease ignore the symptoms that appear, despite general malaise. It happens that a diagnosis is made only when another disease, such as a severe cold, flu, stress or an accident, causes a sharp deterioration in health.

Sometimes the first sign of the disease is the so-called Addison’s crisis, or acute adrenal insufficiency. The crisis is manifested by severe abdominal and lower back pain, muscle weakness, loss of consciousness or unmanageable sleepiness, low blood pressure, dehydration, severe diarrhea and vomiting. Sometimes a shock develops as a consequence of not delivering the right amount of blood to individual organs. This situation always requires urgent medical attention. Otherwise, the patient may die.

Diagnosis of the disease

Often the first signal for testing for Addison’s disease is darkening of the skin. Sometimes the search is caused by abnormal results of general checkups, such as elevated potassium levels, low sodium levels, unexpected changes in ECG or chest X-rays. Of course, such deviations from the norm may be caused by other diseases. Therefore, further tests should be performed, e.g. the level of cortisol and aldosterone in the blood and urine, and the hormone ACTH. It is also important for the patient’s future to identify the cause of adrenal insufficiency.

Treatment principles

Since Addison’s disease is caused by a lack of cortisol and aldosterone, treatment is about making up for them. Therapy must be continued for the rest of your life. Natural cortisol is replaced with hydrocortisone or cortisol acetate, less often with Prednisone. Medicines should be taken 2 times a day – in the morning and in the afternoon. The aldosterone deficiency is corrected by the administration of the synthetic steroid fludrocortisone. The dose of the drug is selected according to the height and weight of the patient.

Proper therapy also includes salt and fluid replenishment. During the weakening of the body, e.g. due to a cold with high fever, the doctor prescribes additional doses of hydrocortisone.

How to cope?

Addison’s disease is a chronic disease and, like any disease of this type, it significantly influences the change of lifestyle, plans and expectations. However, it is worth remembering that correctly selected and regularly taken doses of hormones guarantee a normal life and do not affect its duration. If the patient is under the care of an endocrinologist, he does not have to give up his plans, because taking medications does not limit his physical, mental or professional possibilities.

Addison’s disease does not exclude motherhood either, but pregnancy must be carefully managed, taking into account the woman’s hormonal needs.

Every ill person suffering from Addison’s disease should – like people suffering from diabetes – wear a bracelet or pendant with information about the disease so that in the event of the crisis described above, the doctor can take appropriate action immediately.

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