Addisson disease

Hypocorticism, as the disease is otherwise called, is characterized by insufficient activity of the adrenal cortex and thus insufficient levels of hormones produced here. And these are the hormones  cortisol and  aldosterone.

This form is also called primary adrenal insufficiency.

That production was insufficient, it must be damaged to nearly 90 percent of the adrenal cortex. So this is a serious failure of the organ to function. If this condition is not treated, the body can be fatal due to the absence of these hormones.

Secondary adrenal insufficiency is caused by insufficient hormonal production of the pituitary gland. The pituitary gland produces a hormone that affects the function of the adrenal cortex. This hormone is called ACTH, and therefore an adrenocorticotropic hormone.

There is also a so-called iatrogenic form, which is caused by a decrease in adrenal function. This condition is temporary. And it is caused by long-term administration of drugs, namely glucocorticoids.

The disease is not very common. It affects about one person out of 25,000 people in the population. The disease most often breaks out between the ages of 30 and 50. It affects both men and women.

The disease can occur based on hereditary predispositions. But it can also occur during life due to several risk factors. These can cause destruction of the adrenal glands. This is caused, for example, by inflammation, metabolic disorders or overuse of certain medicines.

What are adrenals?

The adrenal glands are a paired organ. They are located on the upper side of the kidneys. Where the kidneys also insist. Their name also follows from this storage. The adrenal glands belong to the endocrine glands. This means that they secrete hormones into the bloodstream and thus into the whole body.

The adrenal glands consist of bark and marrow. And, each of them has its own function:

• the adrenal cortex makes up about 70%, it produces steroids (corticosteroids)
  • mineralocorticosteroids, ie cortisol, regulate the metabolism of sugars and proteins
  • Glucocorticosteroids, or aldosterone, regulate minerals and body water
  • the bark also produces small amounts of sex hormones
• the marrow produces catecholamines, namely adrenaline and norepinephrine

The cause of Addison's disease is insufficient production of hormones in the adrenal cortex. And so the hormones  cortisol and  aldosterone. It is also referred to as primary adrenal insufficiency, but also as a peripheral form. 

Adrenal function is most often impaired due to the autoimmune process, in up to 80 percent of cases. Sometimes poor function is caused by inflammation. And that can be TB, or tuberculosis.

Another case is meningococcal infection and a serious life-threatening condition in Waterhouse-Friderichse syndrome. Which is a rare complication of meningococcal infection. It causes a shock condition that can lead to organ failure.

Another less common reason is the tumor, but also the metastasis of another nation to the adrenal glands. It is also caused by adrenal bleeding and AIDS, for example. Genetic predisposition (HLA D3) may also have an effect.

The central form is the one that has its cause in the hypothalamus. It is also referred to as secondary adrenal insufficiency. The adrenal glands are fine, but they lack regulation by the hormone ACTH. The cause of this form can be a tumor, injury, infection or pituitary dwarfism.

Addison's disease is manifested by weakness, fatigue and various mental disorders, such as behavioral disorders or depression, memory disorders, restlessness. Insomnia may also be present. This is mainly due to the absence of hormones in the body, which have a significant effect on these activities.

Increased pigmentation throughout the body is also a typical manifestation. The skin may appear more tanned, even without previous sun exposure, even in winter. The cause is increased production of CRH and ACTH, which in turn causes increased production of hyperpigmentation-promoting substances.

However, it is most visible in places that are more pigmented even in the case of a healthy individual. These are mainly the  areas around the anus, around the genitals, around the breasts, on the palms, on the hands or on the face. There may also be so-called graphite spots in the oral cavity in the area of ​​the first upper stools. Anorexia, nausea, vomiting, abdominal pain and diarrhea are also typical.

Decreased blood pressure may also be present, creating a risk of collapse and therefore injury. And women can experience menstrual disorders or their complete absence. Dehydration and weight loss also occur when aldosterone is deficient. The central form has the same manifestations, but it does not stain the skin, mucous membranes and hyperpigmentation. Manifestations are generally milder in the central type of disease.

Summary of symptoms in Addisne's disease:

• general fatigue
• low blood pressure, ie hypotension
• weight loss
• hyperpigmentation of the skin, face, skin folds, palms, breast area
• graphite spots on the mucosa in the oral cavity
• thirst
• increased appetite for salty
• stomach ache
• diarrhea, for increased levels of potassium in the blood
• heart rhythm problems cause excessive levels of potassium in the blood
• mental disorders
• joint and muscle pain
• dizziness and a feeling of instability

In Addison's disease, a critical deficiency of hormones can occur, which is typically manifested by an Addison's crisis, which may also be referred to as an adrenocortical crisis. It is a condition that can endanger a person's life. The crisis manifests itself as follows:

• weakness
• apatiou
• confusion
• fever
• fluid loss to dehydration
• hypotension
• tachycardia, increased pulse
• in the blood it is proved, for example:
  • hyperkalaemia - increased potassium levels
  • hyponatraemia - decreased sodium levels
  • hypoglycaemia - low sugar
• nausea, ie the feeling of vomiting to vomiting
• excessive taste of salty

Diagnosis is based on the evaluation of clinical symptoms. Such as fatigue, weakness, nausea, loss of appetite, weight loss or abdominal pain. In addition, an increased appetite for salty and, of course, the presence of hyperpigmentation. Blood laboratory tests are used to confirm the diagnosis.

The level of hormones and ions (sodium, potassium) is assessed in the blood. Decreased levels of cortisol and aldosterone are detected. ACTH will be reduced for the central type. In the peripheral form, the level of ACTH is increased. Other parameters in the blood are then evaluated.

Decreased glucose or sodium levels are also present in Addison's disease. Conversely, potassium levels are elevated due to decreased aldosterone levels. In the autoimmune cause, the level of antibodies (against 21-hydroxylase is increased, up to 90 percent of those affected). 

CT of the abdominal cavity and  adrenal glands or magnetic resonance imaging (MRI) of the pituitary gland are also used in the examination. USG, ie ultrasononography of the adrenal glands, is helpful.

The disease usually begins after 30 years of age and due to the reduced level of adrenal hormones, a person suffers from general fatigue , malaise. Associating the loss of appetite, nausea, weight loss, as well as the presence of anorexia.

Diarrhea occurs with elevated potassium levels, but the risk of heart rhythm is more serious. Symptoms include abdominal pain and increased appetite for salt and thirst, as a result of reduced sodium levels. This can cause dehydration, hypotension. Low blood pressure is consequently a risk of collapse, and therefore of injury. 

Increased pigmentation also occurs as a result of increased production of hormones (ACTH and CRH, which is corticoliberin). These in turn cause increased levels of other substances that cause this hyperpigmentation. One gives the impression of a tan, even without previous sunbathing, even in winter.

Pigmentation is increased mainly on the face, in the skin folds and lines, in places such as hands, palms, knees, elbows, but also in the area of ​​the nipples. Gray graphite spots form on the mucous membrane of the oral cavity. However, these cutaneous manifestations do not occur in the central form. In general, the symptoms are milder.

In addition, there are psychological changes in Addison's disease. And these can be behavioral disorders, the mentioned anorexia, depression, but also memory disorders or general restlessness. Fatigue is exacerbated by insomnia. In women, dysmenorrhea is associated, which is a disorder of menstruation to its absence.

The Addison crisis is a severe form of hormone deficiency. In the acute type, it can endanger a person's health and life. Children are particularly at risk. Manifestations are exacerbated by the feeling of vomiting or vomiting, dehydration, hypotension, confusion.

Elevated potassium levels, ie hyperkalemia, cause diarrhea, which worsens dehydration and hypotension. However, the greatest risk of hyperkalemia is heart rhythm problems. The body is at risk of hypoglycemia, shock, failure of various organs or circulatory failure.