Synonyms in the broadest sense
- Adrenocortical insufficiency
- Primary adrenocortical insufficiency
- Addison’s Disease
- Addison’s Syndrome
Definition and introduction
Addison’s disease is a functional disorder of the adrenal cortex. It is also known as primary adrenal cortex insufficiency and is a rare disease. However, if Addison’s disease remains untreated, it is fatal and thus has clear clinical relevance.
In most cases, autoimmune processes destroy the cells of the adrenal cortex. Addison’s disease can be asymptomatic for many years and only manifests itself in special situations. The spectrum of symptoms of Addison’s disease is broad. Thanks to modern medications, the quality of life is high and life expectancy normal today, provided that the patient is well trained and willing to cooperate (compliance).
Frequency
Women are more frequently affected by Addison’s disease than men. The mean age of onset of the disease is about 40 years. However, there is a clear spread. With a prevalence of about 1-5 patients per 100 000 inhabitants, primary adrenal insufficiency is a rather rare disease.
Classification of Addison’s disease
Addison’s disease is classified according to the course of the disease on the one hand: on the other hand, it is classified according to the causes. The most common triggering factors are therefore
- Slowly Progressive
- Acute with rapid loss of adrenal cortex function
- Autoimmune processes (70 – 80 %)
- Infections
- Infarcts
- Tumors
- Other
Causes and development
Addison’s disease is caused by a defensive reaction of the body’s immune system against the body’s own cells. These autoantibodies are specifically directed against the cells of the adrenal cortex. This is followed by a defensive reaction and thus a destruction of the cells.
This form of primary adrenal insufficiency, in which antibodies formed by the body itself and directed against the body’s own structures cause destruction of the adrenal cortex, is the most common form of primary adrenal insufficiency, accounting for 70-80% of cases. Primary adrenal insufficiency can also be caused by:. Other causes of Addison’s disease are:
- Tumors and their metastases,
- Infarcts (Waterhouse-Friderichsen-Syndorm) and
- Infections (e.g. tuberculosis, HIV/AIDS, cytomegalovirus)
- Sarcoidosis
- Amyloidosis (abnormal deposition of proteins between cells)
- Hemochromatosis (iron storage disease)
- Adrenoleukodystrophy (hereditary disease manifesting in childhood and characterized by rapid neurological decay)
- Or even bleeding or
- Certain drugs.
Due to stress, healthy people normally release cortisol, which is therefore often called stress hormone.
With an existing adrenal insufficiency, the body is no longer able to maintain cortisol production, let alone increase it. The body gets into a state of hypocortisolism – a low cortisol level. Particularly in people with undiagnosed adrenal insufficiency, stress and the hypocortisolism associated with it can lead to the discovery of the disease or, in the worst case, to an Addison crisis.
This is a life-threatening condition with various symptoms such as clouding of consciousness, hypoglycemia, fever, vomiting, etc. which requires immediate emergency medical treatment. Hashimoto thyroiditis is a chronic inflammation of the thyroid gland caused by autoimmune antibodies.
The body’s own cells are directed against the thyroid gland due to as yet unexplained causes and lead to cell death in the course of the disease. Those affected then show an underactive thyroid gland. Adrenal insufficiency is often associated with other diseases, such as Hashimoto’s thyroiditis.
In this context, doctors also like to speak of a Pluriglandular Syndrome, i.e. a disorder of several organs with glandular function, which serve the production of hormones. The exact cause is still unknown. A genetic component is suspected, but has not yet been clearly proven by scientific studies.For patients suffering from Addison’s disease, regular checks of the organs are important in order to quickly detect a possible loss of function and to be able to treat it properly.
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