Brief overview
- Symptoms: Browning of the skin, fatigue and listlessness, low blood pressure, weight loss, fluid deficiency.
- Course of disease and prognosis: Treated, life expectancy is normal; untreated, the disease is fatal. In stressful situations, it is important to adjust the hormone dose to prevent a life-threatening Addisonian crisis.
- Diagnosis: Various laboratory tests, control of cortisone and ACTH levels, ACTH stimulation test, imaging techniques.
- Treatment: Lifelong intake of the missing hormones
What is Addison’s disease?
While the so-called catecholamines, first and foremost adrenaline and noradrenaline, are produced in the medulla, the cortex produces the androgens (sex hormones) as well as aldosterone and cortisol in two different zones.
To understand how the typical symptoms of Addison’s disease occur, it is important to understand the functions of the adrenocortical hormones:
Cortisol also influences the digestive tract (appetite), the sex drive and the psyche. In higher concentrations, the hormone has an anti-inflammatory effect by regulating the body’s immune defenses. Doctors take advantage of this, for example, to combat allergic symptoms or autoimmune diseases.
Androgens include hormones that are converted in body tissue to the sex hormones testosterone or estrogen. The male sex hormone testosterone is also secreted in small quantities by the adrenal cortex in women. In women, the androgens cause pubic hair to grow, among other things.
Regulatory circuit of hormones
The hypothalamus responds by sending signals to release certain hormones. For example, in the event of a lack of cortisol, the hypothalamus releases the messenger substance CRH (corticotropin releasing hormone). This causes the pituitary gland to release ACTH (adrenocorticotropic hormone). ACTH, in turn, travels through the blood to the adrenal cortex, where it transmits the signal for cortisol release.
Forms of Addison’s disease
What are the symptoms of Addison’s disease?
In primary adrenocortical insufficiency, if the adrenal gland itself is affected by disease, about 90 percent of the cortex has already been destroyed by the time the typical symptoms occur. The following leading symptoms are present in more than 90 percent of those affected:
Low blood pressure: Due to the absence of the hormone aldosterone, which regulates mineral and thus water balance, blood pressure drops to circulatory failure.
Other symptoms include:
- General exhaustion and severe listlessness (adynamia)
- @ Weight loss and lack of fluids (dehydration)
Weight gain is not a typical symptom of Addison’s disease, but is a possible side effect of glucocorticoid therapy.
- Hunger for salty foods
- tiredness, fatigue
- Digestive problems such as nausea, vomiting or abdominal pain
- Absence of pubic hair in women, potency problems in men
- Psychological problems such as depression, irritability and apathy
- In infants, growth retardation
Particularly in the case of slowly progressing primary Addison’s disease, it happens that sufferers and doctors misinterpret the gradually developing complaints and interpret them as exhaustion syndrome or symptoms of old age. The multitude of life-threatening symptoms shows how important the hormones are, which achieve enormous effects even in tiny concentrations.
Symptoms of the Addison crisis
- Drop in blood pressure
- Massive loss of fluid through the kidneys and threat of dehydration of the body
- Fever
- Circulatory collapse up to shock and circulatory failure
- Dangerously low blood sugar (hypoglycemia)
- Severe abdominal pain
Physicians often first discover an existing adrenocortical insufficiency due to an Addisonian crisis. The acute life-threatening condition can only be averted with a high-dose administration of hydrocortisone. The emergency physician must act quickly here!
Problems are possible if the hormone concentration is not adjusted to a currently prevailing stress situation. In this case, an Addison’s crisis may occur, which is life-threatening if medical treatment is not received in time. If left untreated, Addison’s disease is always fatal.
Causes and risk factors
Important causes of primary adrenocortical insufficiency, in which the adrenal gland itself is diseased, include:
- Autoimmune adrenalitis: this disease is the most common cause, accounting for about 80 percent. The body’s own cells target the tissue of the adrenal gland and slowly destroy it. Addison’s disease is often associated with other autoimmune diseases, for example, chronic thyroiditis (Hashimoto’s thyroiditis).
- Infections: Infections with certain pathogens are responsible for destruction of the adrenal gland in some cases. In the age of tuberculosis, this was the main cause of Addison’s disease. However, histoplasmosis, AIDS, or cytomegalovirus also sometimes lead to Addison’s disease.
- Bleeding:Blood thinning agents in rare cases cause bleeding into the adrenal glands and may severely damage them.
- Heredity: Congenital adrenal hypoplasia, for example, is congenital. The underdevelopment of the adrenal gland leads to severe symptoms already in infancy.
Examinations and diagnosis
The physician first detects typical symptoms of Addison’s disease in a detailed discussion with the affected person. The specialized endocrinologist uses this information on the medical history to make an initial assessment.
A so-called ACTH stimulation test helps the doctor to find the cause of Addison’s disease: For this purpose, the affected person receives the pituitary hormone ACTH via the vein. The doctor then determines the level of cortisol in the blood. If it rises, the adrenal gland is still functioning and the cause is most likely in the pituitary gland. If, on the other hand, the cortisol level remains low despite ACTH administration, there is probably a primary adrenal insufficiency.
Treatment
The only treatment for primary and secondary Addison’s disease is the lifelong intake of the missing hormones (substitution therapy). Affected patients usually take hydrocortisol in combination with fludrocortisol two to three times a day. In women who experience a loss of libido, treatment with another hormone (dehydroepiandrosterone, DEHA) is possible.
Addison’s disease, with the exception of tertiary adrenocortical insufficiency, is incurable. Affected individuals therefore require hormone therapy for the rest of their lives, as the disease is fatal if left untreated. A special diet is not required for Addison’s disease.
