Addison's disease

Addison's disease is a rare but severe adrenal disorder. Read how to recognize and treat addison's disease!

Addison's disease

Addison's disease (also Addison's disease) is a serious, fortunately rare disease. The adrenal cortex produces too little of certain vital messengers. The treacherous: For sufferers, the skin tints brownish, making them even in the early stages of the disease even a particularly healthy impression. Nevertheless, Addison's disease is lethal if left untreated. Particularly dangerous is a so-called Addison crisis, Read all important information about Addison's Disease below.

ICD codes for this disease: ICD codes are internationally valid medical diagnosis codes. They are found e.g. in doctor's letters or on incapacity certificates. E27E35E23

Product Overview

Addison's disease

  • description

  • symptoms

  • Causes and risk factors

  • Examinations and diagnosis

  • treatment

  • Disease course and prognosis

Addison's disease: description

Addison's disease arises when it becomes one Lack of certain hormones (Messenger substances) from the Adrenal cortex comes. The adrenal gland is the educational site of some vital messenger substances. It sits cap-shaped on the kidney and divides below its protective capsule into the adrenal medulla (NNM) and the adrenal cortex (NNR). While in the marrow the so-called catecholamines, especially adrenaline and norepinephrine, are released, the bark produces the hormones in two different zones aldosterone, cortisol and androgens (Sex hormones).

To understand the typical symptoms of Addison's disease, one needs to understand the functions of the adrenocortical hormones:

  • The cortisol belongs to the group of glucocorticoids, This term includes a part of the word "glucose", ie sugar, on which the cortisol has a decisive effect: in stressful situations, the hormone stimulates metabolic processes that provide energy in the form of sugar. For this, however, proteins, which are mainly in the muscle tissue, consumed, and created fat as emergency reserves. In addition, cortisol still has a significant effect on the metabolism of bones, skin, muscles and connective tissue. Cortisol also affects 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 defense. This is used, for example, in combating allergic symptoms or autoimmune diseases in medicine.
  • The aldosterone is a so-called mineralocorticoid, If this hormone is missing, the human is not viable. In the kidney, aldosterone regulates the retention of sodium and water and is therefore vital for maintaining blood pressure and regulating mineral metabolism.
  • To the androgens These include hormones that are converted into the sex hormones testosterone or estrogen in body tissues. The male sex hormone testosterone is released in small amounts in women from the adrenal cortex. Among other things, androgens cause a growth of pubic hair in women. Therefore adrenal insufficiency leads to a loss of pubic hair.

Loop of hormones

Whether the adrenal cortex releases hormones and how much is subject to a finely-regulated body control mechanism in which the brain plays the central role. The all-supervising organ, also called "master gland" is the hypothalamuswho is sitting deep in the brain. Information from the various measuring stations in the body on blood sugar, blood pressure, temperature and much more converge in the hypothalamus, which can then send the signal to release certain hormones. Thus, the hypothalamus responds to a lack of cortisol with the release of the messengerCTH (Corticotropin Releseaing Hormones), which first at the Pituitary gland (pituitary) for the distribution of ACTH (adrenocorticotropic hormone) leads. The ACTH then passes through the blood to the adrenal cortex, where it transmits the signal for cortisol release.

Forms of Addison's disease

A deficiency of the adrenal cortex hormone may occur when the adrenal gland itself is affected by a disease (primary adrenocortical insufficiency), or when the superordinate regulatory centers in the brain are disturbed (secondary or tertiary Adrenocortical insufficiency).

Addison's disease: symptoms

If the adrenal gland itself is affected by a disease, about 90 percent of the bark must already be destroyed, until it comes to the first symptoms. Especially with a slowly progressing primary Addison's disease, the symptoms can be misinterpreted and interpreted as fatigue syndrome or signs of aging. The multitude of life-threatening symptoms shows the importance of hormones, which produce enormous effects even in tiny concentrations.

In the primary adrenocortical insufficiency it comes to the typical symptoms of Addison's disease:

  • Browning of the skin: The darker tint of the skin helped the disease to the nickname "bronze disease". The pituitary hormone ACTH, which is released more frequently in the event of a cortisol deficiency, causes an increased release of the melatonin. This leads, inter alia, to the browning of the skin, which is particularly visible in the oral mucosa and the skin lines. This symptom occurs only in the primary Addison disease.
  • salt hunger: The lack of aldosterone causes a hunger for salty foods.
  • Low blood pressure: Due to the absence of the hormone aldosterone, which regulates the mineral and thus the water balance, the blood pressure drops to circulatory failure.

In addition to the typical symptoms of Addison's disease, the many functions of cortisol are also noticeable on other levels:

  • Fatigue and exhaustion
  • Loss of appetite and weight loss
  • Indigestion such as nausea, vomiting or abdominal pain.
  • Women may experience a lack of pubic hair while men are confronted with potency issues.
  • Mentally, patients often suffer from depression, irritability and apathy.
  • In infants it comes in addition to a growth stop. Dangerous Addison Crisis

Already existing adrenal gland disease can lead to a so-called Addison crisis in mental or physical stress situations, in case of injuries or inadequate medication. The sudden onset of hormonal deficiency (below demand) leads to life-threatening symptoms:

  • Drop in blood pressure
  • massive fluid loss through the kidneys and impending dehydration of the body
  • fever
  • Circulatory collapse until shock and complete circulatory failure
  • dangerously low blood sugar (hypoglycaemia)
  • Severe abdominal pain similar to the so-called acute abdomen

Often the existing adrenocortical insufficiency is first detected by an Addison crisis. The acutely life-threatening condition can only be averted with a high dose of hydrocortisone. The ambulance has to act fast here!

Addison's Disease: Causes and Risk Factors

Addison's disease is caused by a life-threatening hormone deficiency. Disorders of the adrenal gland itself, or the superordinate switching points in the brain, the pituitary gland or the hypothalamus can be the cause of Addison's disease.

When the adrenal gland is diseased, slow tissue loss often leads to gradually increasing symptoms that are often not recognized at first. Causes of these so-called primary adrenocortical insufficiency can be:

  • Autoimmune diseases: They are the most common cause of adrenocortical insufficiency. The body's own cells are directed against the adrenal tissue and destroy it slowly. Women are more frequently affected by this form, whose cause is unknown, than men.

  • Waterhouse-Friderichsen syndrome: In a serious blood poisoningMeningococcal infarction may cause infarction of the adrenals. The adrenals are not adequately supplied with blood and suddenly fail.
  • infections: Even infections with foreign pathogens may be responsible for the destruction of the adrenal gland. In the age of tuberculosis This was the main cause of Addison's disease. But also histoplasmosis, AIDS or that cytomegalovirus can lead to Addison's disease.
  • Addison crisis: Injuries or special stress situations can exacerbate an existing adrenal insufficiency to a complete failure of hormone production. The symptoms are life-threatening and need to be treated by the doctor.
  • Nebennierenhämorrhagie:Blood thinning agents In rare cases, they can lead to bleeding into the adrenal glands and severely damage them.
  • tumors: Very rarely tumors of the adrenal cortex are responsible for their functional failure.
  • heredityCongenital adrenal hypoplasia, which leads to the most severe symptoms in infancy, is innate, for example.

Also malfunctions of the superior regulation in the brain, the pituitary or of hypothalamus can cause Addison's disease. To this form of secondary adrenocortical insufficiency Damage to these brain areas after stroke, tumors, skull injury or brain surgery is very rare.

to tertiary adrenocortical insufficiency It comes from a long-term intake of high-dose cortisone preparations. This suppresses the formation of the hormone CRH in the hypothalamus. The downstream pituitary gland produces less ACTH and the adrenal cortex therefore slows down hormone production. If the cortisone intake is interrupted, the adrenal cortex begins slowly with the production of hormones, so that it is initially a deficiency. Because of this, cortisone medicines should always be phased out gradually and never stopped abruptly.

Addison's Disease: Examinations and Diagnosis

Typical Addison's symptoms are first tracked down in a thorough patient survey. The specialized endocrinologist can use this information to gain an initial assessment.

The suspicion of adrenocortical insufficiency is with blood tests and laboratory tests investigated. In the foreground are, in addition to the usual blood parameters, the hormone levels of cortisol from the adrenal cortex and the ACTH of the pituitary gland. The cortisol levels are markedly reduced in acute Addison's Disease, but may be normal in the morning when the disease is chronic. Therefore, in addition one 24-hour follow-up of urine makes sense.

A so-called ACTH stimulation test helps to find the cause: The pituitary hormone ACTH is supplied from the outside and then determines the cortisol level in the blood. If it increases, the adrenal gland is still functional and the cause is most likely in the pituitary gland. However, if the cortisol level remains low despite ACTH administration, primary adrenocortical insufficiency is likely to be present.

Next can be a special Antibody test to detect an autoimmune disease. Imaging procedures like the Computer or magnetic resonance tomography or Ultrasonic One makes use of the finding of tumors or infarcts.

Addison's disease: treatment

The only therapy of a primary and secondary Addison's disease, in which the adrenal cortex has already lost at least 90 percent of its function, is the lifelong intake of missing hormones (Replacement therapy). Hydro cortisol in combination with Fludrocortisol This is taken three times a day. In stress situations, the dose must be adjusted by the doctor so that it does not come to a deficiency.

Addison's disease, with the exception of tertiary adrenal insufficiency, is incurable - patients are required to take hormones throughout their lives.

Read more about the investigations

  • blood collection
  • blood test

Addison's Disease: Disease Course and Prognosis

If a slow-onset, chronic Addison's disease is recognized early enough, it can be treated well by taking the vital hormones. Despite lifelong hormone replacement, those affected can live a normal life with an average lifespan. Problems can occur if the hormone concentration is not adjusted to a prevailing stress situation. If Addison's disease is not treated, the disease is always fatal.

Life-threatening is an acute one Addison crisis - especially in connection with the Waterhouse-Friderichsen syndrome.

These laboratory values ​​are important

  • ACTH
  • adrenaline

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