Addison’s disease is a disease in which the adrenal glands, sitting on the kidneys, cannot produce enough cortisol and aldosterone hormones. You can find more information below.
What is Addison’s disease?
Addison’s disease is a gradual, progressive dysfunction of the adrenal cortex. It causes characteristic symptoms such as hypotension, hyperpigmentation and can lead to an adrenergic crisis with cardiovascular circulatory collapse. Diagnosis is made on a clinical basis and by measuring the elevated adrenocorticotropic hormone (ACTH) level at low cortisol levels. Treatment usually consists of hydrocortisone substitution. Sometimes other hormones are also substituted. This disease is also known as primary adrenal insufficiency .
How common is it?
Thousands of people around the world are affected by this disease. It occurs equally in both men and women and in all age groups, but is most common in the 30-50 age range.
What causes Addison’s disease?
Addison’s disease is usually caused by destruction of the adrenal cortex tissue. In the majority of cases, data show that between 70 and 90 percent this disease is caused by an autoimmune disease . This is a disease in which the immune system, that is, the body’s own defense, attacks the body’s own cells.
In Addison’s disease, components of the immune system are directed against the adrenal cortex and antibodies can be detected in the blood. Addison’s disease may be accompanied by other autoimmune diseases, such as autoimmune inflammation of the thyroid gland (usually Hashimoto’s disease ) or diabetes .
Rarely, inflammation of the blood vessels that supply the adrenal glands, as well as infectious diseases such as AIDS or tuberculosis, may be responsible for the destruction of the adrenal cortex. In addition, benign or malignant tumors (eg, adrenal tumor ) and inherited diseases are also possible causes in a few cases.
Another rare cause of underactive adrenal cortex is malfunction in the brain. This can happen if the pituitary gland or hypothalamus is not working properly (for example, due to a tumor) and consequently does not stimulate the adrenal cortex to produce hormones.
Long-term use of a cortisone preparation may also decrease hormone production in the adrenal cortex. Therefore, cortisone as a drug should never be stopped abruptly, but should be reduced slowly, i.e. discontinued by reducing the dose for a long time. This gives the adrenal cortex enough time to resume its function.
What are the symptoms of Addison’s disease?
Due to the lack of these hormones in the body, Addison’s disease causes general weakness and circulatory problems such as rapid fatigue, weight loss, low blood pressure (hypotension), and a tendency to feel dizzy and collapsed . You may also experience nausea and vomiting, abdominal pain , rapid heartbeat, and irregular heartbeat . Some patients develop a ” hunger for salt ” or a craving for unusual foods such as salt water.
Browning of the skin is typical, particularly evident on the soles of the feet, palms, scars and oral mucosa. For this reason, Addison’s disease is also called “bronze skin disease”. In addition to this brown pigmentation of the skin, Addison’s patients often also experience depigmentation or white spot disease ( vitiligo ).
Women’s menstrual periods may be delayed and they may lose interest in sex, men may suffer from erectile dysfunction.
The so-called Addisonian crisis is often sufficient to make a diagnosis. The crisis results from physical stress, for example in the context of infections, diarrhea or accidents . In these cases, the need for a large number of hormones in the body increases. The sudden lack of hormones in an Addisonian crisis causes severe circulatory disorders and even shock.
The symptoms of this crisis are:
- sudden drop in blood pressure
- peritonitis abdominal pain
- urinary retention
- Possibly diarrhea and vomiting
- low blood sugar (hypoglycemia)
- Acidification of the blood (acidosis)
Addisonian crisis is a life-threatening condition that requires prompt treatment.
How is Addison’s disease diagnosed?
If Addison’s disease is suspected, a brief ACTH (adrenocorticotropic hormone) test is done. ACTH is produced in the pituitary gland. It mainly promotes the formation of glucocorticoids (for example, cortisol) in the adrenal cortex.
Performing the ACTH short test
After the cortisol level in the blood is determined, a certain amount of ACTH is injected into the vein. After 30 and 60 minutes, the amount of cortisol in the blood is measured again. A normal increase in the hormone indicates a functioning adrenal cortex. The lack of increase indicates Addison’s disease.
Other blood tests help narrow down the extent of the disease more precisely. Other hormones of the adrenal cortex, white and red blood cells, kidney and thyroid values, and electrolytes (sodium, potassium) are determined in this way. To detect an autoimmune disease, autoantibodies against the adrenal cortex are sought. The urinary excretion of cortisol and aldosterone can also be measured.
Imaging methods such as sonography (ultrasound) , computed tomography , or magnetic resonance imaging can be used to visualize changes in the adrenal glands, such as an enlargement or tumor.
How is Addison’s disease treated?
The most important treatment principle for Addison’s disease is usually lifelong replacement of the missing hormones. For example, glucocorticoids can be replaced with hydrocortisone, mineralocorticoids with fludrocortisone.
Substitution of androgen hormones is currently being considered, but is not yet part of standard therapy. According to research, those who take additional androgens (especially women) have improved mental well-being.
Affected patients usually take hydrocortisone in tablet form in two to three doses throughout the day . It is not uncommon for them to take higher doses in the morning than later in the day, because the natural level of glucocorticoids is highest in the morning and the treatment should be adjusted to the natural hormone level.
In special stressful situations, the dose should be adjusted by the doctor to avoid a deficiency. Possible side effects of an overdose of hormones are especially muscle weakness, fat distribution disorders, skin changes, osteoporosis (bone loss) and depression.
In stressful situations , care should be taken to increase the cortisone dose, otherwise a deficiency situation may occur that triggers an Addisonian crisis. Such stressful situations are, for example, febrile infections, gastrointestinal diseases, surgery or dental treatment.
The doctor will regularly check the correct dosage of fludrocortisone taken once a day (in the morning) according to the level of potassium or renin in the blood, body weight, blood pressure and heart rate.
Renin is a messenger that plays an important role in regulating aldosterone release. An overdose of fludrocortisone is manifested by headache, high blood pressure, weight gain, edema (water accumulation in tissue), cardiac arrhythmia, weakness, and confusion.
People suffering from Addison’s disease should always carry with them an emergency identification card with information about the disease and the current dosage of their medication. Addison patients should also always have an emergency kit available, such as cortisone suppositories or hydrocortisone ampoules for injection.
If Addison’s disease is caused by an infection or a tumor of the adrenal cortex, additional treatment of the triggering disease, such as antibiotics for bacterial infections or surgical removal of a tumor, is important.
Your doctor will decide which treatment you should receive.
Outlook in Addison’s disease
If Addison’s disease is recognized in time and treated appropriately by replacing the missing hormones, the life expectancy of those affected is not restricted. They can live a normal life. Quality of life may be somewhat affected by the need to take the tablets.
In women with Addison’s disease and during pregnancy , it is possible that there are usually no complications if the dose of the hormones taken is adjusted.
It is important to increase the dose of glucocorticoids in all stressful situations, especially after accidents, illness and operations. Adjusting the hormone concentration to an already prevailing stress state is important to avoid Addisonian crisis.
If a serious infectious disease or malignant disease is the cause of Addison’s disease, the prognosis and life expectancy of the individual concerned depends on the underlying disease.