In Addison’s disease, the production of vital hormones in the adrenal cortex is disrupted. The disease is insidious and can lead to death if left untreated. Which symptoms indicate Addison’s disease and which therapy helps.
In Addison’s disease (also known as Addison’s disease, bronchial skin disease or salt hunger disease), the adrenal cortex produces fewer and fewer hormones. Doctors speak of insufficiency, i.e. weakness or underfunction of the adrenal cortex. The disease is rare, occurs in all age groups, and affects both sexes equally often.
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Everyone has two adrenal glands. The small organs lie above the kidneys and consist of an inner adrenal medulla and an outer adrenal cortex. The cortex of the adrenal glands produce hormones which, as messenger substances, perform vital tasks in the human body. Doctors differentiate between three groups of hormones:
- Glucocorticoids like cortisone boost the energy supply in stressful situations, have an anti-inflammatory effect and suppress the body’s own defenses. They are important for the metabolism of bones, skin, muscles and connective tissue. They also affect appetite, sex drive and psyche.
- Mineralocorticoids like aldosterone regulate the body’s salt and water balance and thus control blood volume and blood pressure.
- Androgens like testosterone, affect bone growth, increase muscle mass, lower cholesterol and increase protein build-up. They also control the development of male sexual characteristics and the formation of semen. In men, in addition to the adrenal cortex, the testes also produce androgens.
Causes of Adrenal Weakness
Most often, the adrenal cortex is underactive because its tissues are destroyed (primary adrenal insufficiency). Strictly speaking, the term “Addison’s disease” only refers to this primary form of adrenal weakness.
Most of the people affected are atrophy of tissue without any identifiable cause. Doctors suspect a false reaction of the immune system, in which the body’s own defense attacks the cells of the adrenal glands. Other possible causes are infections, inherited diseases, tumors or bleeding into the adrenal glands (for example after years of taking anticoagulant medication).
The brain controls the adrenal cortex via messenger substances such as CRH (corticotropin-releasing hormone) or ACTH (adrenocorticotropic hormone). Therefore, malfunctions of the brain can also inhibit the production of hormones in the adrenal cortex, for example after a stroke, in the case of brain tumors, injuries or operations (secondary adrenal insufficiency).
Long-term intake of high-dose cortisone preparations also suppresses the formation of messenger substances in the brain that stimulate hormone production in the adrenal cortex (tertiary adrenal insufficiency). After stopping cortisone therapy, the adrenal cortex starts producing hormones again extremely slowly. It is therefore important to taper off high-dose cortisone intake very slowly.
Symptoms of hypofunction in Addison’s disease
A deficiency in adrenal cortex hormones usually develops slowly. Many people with impaired function of the adrenal cortex have no symptoms under normal living conditions. Only significant physical and physical stress leads to symptoms such as operations, infections, burns or a serious illness. Persistent signs of the disease probably only appear when at least 90 percent of the adrenal cortex tissue has already been destroyed.
Typical symptoms of Addison’s disease are:
- Hyperpigmentation (brown discoloration) of the skin and mucous membranes: The body reacts to the lack of cortisol with an increased production of melatonin, which is responsible for the pigmentation of the skin. The skin discolours particularly in the area of skin folds, scars, nipples, on the extensor sides of arms and legs, over bony elevations and in places that are exposed to the sun. Black spots may appear on the forehead, neck, face and shoulders. In exceptional cases, Addison’s disease can also lead to white spot disease (vitiligo).
- Salt hunger: The lack of aldosterone causes cravings for salty foods and drinks.
- Low blood pressure (hypotension): Due to the aldosterone deficiency, the blood pressure drops – this can lead to circulatory failure.
In addition, the following signs may indicate adrenal insufficiency:
Addison Disease Emergency: The Addison Crisis
The most severe form of the disease is the life-threatening Addison’s crisis. This happens when people with an already damaged adrenal cortex get into a stressful situation or experience severe physical stress. Another possible trigger is the abrupt discontinuation of long-term, high-dose corticosteroid therapy. The hormone level suddenly drops, resulting in severe circulatory disorders up to coma and circulatory failure. If left untreated, the Addison crisis is always fatal.
How does the doctor diagnose Addison’s disease?
The best contact person for Addison’s disease is a specialist in endocrinology, i.e. a doctor who deals with the diseases of hormone-producing glands. If an adrenal insufficiency is suspected, blood and urine tests in particular provide clarity: Among other things, the concentrations of the hormones or their breakdown products are determined.
Doctors use imaging techniques such as ultrasound, x-rays, computed tomography (CT) and magnetic resonance imaging (MRI) to examine the condition of the adrenal glands and the brain and to examine possible tumors, bleeding or signs of inflammation.
Therapy of adrenal insufficiency
There is no cure for primary and secondary adrenal insufficiency. Those affected have to take the missing hormones for life (substitution therapy). The amount of hormones ingested must be set very precisely, and the dose must be adjusted in stressful situations. If the dose is not adjusted or the sick person does not take the medication regularly, an Addison crisis threatens.
In an Addison’s crisis, the patient needs large amounts of adrenal cortex hormones very quickly. Those affected should always have an emergency kit with a hydrocortisone pre-filled syringe and an emergency ID card with them.
Disease course and prognosis in Addison’s disease
The first symptoms of adrenal fatigue are often tiredness and poor performance. Therefore, the disease is initially often misinterpreted as a fatigue syndrome or a symptom of old age and appears for the first time as Addison’s crisis. If the diagnosis is confirmed, thanks to substitution therapy, those affected can usually lead a normal life with an average life expectancy.