In adrenocortical insufficiency, the adrenal cortex can no longer produce enough hormones. A distinction must be made between primary and secondary adrenocortical insufficiency based on location.
What is adrenocortical insufficiency?
Schematic diagram showing the anatomy and structure of the adrenal gland. Click to enlarge. Approximately 5 in 100,000 people suffer from this rare disease. Primary adrenal insufficiency is also known as Addison’s disease. If the adrenal cortex can no longer work properly, then this can lead to a life-threatening situation. The adrenal gland consists of the adrenal cortex, which produces cortisol, aldosterone and sex hormones, and the adrenal medulla, which produces adrenaline and noradrenaline. The hormone ACTH, which is produced in the anterior pituitary of the pituitary gland, controls the production of cortisol. When the level of cortisol in the blood falls, more ACTH is released, stimulating production in the adrenal gland. However, aldosterone, which is responsible for regulating the concentration of potassium and sodium, is also produced in the adrenal cortex. However, unlike cortisol, the production of this hormone is not regulated by the pituitary gland but by the RAAS.
Causes
The causes of developing adrenocortical insufficiency can vary. First, it must be diagnosed whether it is primary or secondary adrenocortical insufficiency. This provides information about the localization of the cause of the disease. In the primary form, the NNR can no longer produce any of the required hormones. It is therefore diseased itself. In the secondary form, the hypothalamus is diseased, which also means that no hormones can be produced. This is simply due to the fact that the hypothalamus no longer sends CRH to the pituitary gland, which in turn then does not send ACTH to the adrenal cortex. ACTH stimulates the production of cortisol. Aldosterone production is only slightly affected.
Symptoms, complaints, and signs
Symptoms of adrenocortical insufficiency usually do not appear until 90 percent of the adrenal cortex has already been destroyed. A distinction is made between general symptoms and specific symptoms. The latter are based on a deficiency of the hormones cortisol and/or aldosterone. General symptoms include nausea and vomiting, loss of appetite, weight loss, fatigue, significantly reduced performance, and nonspecific gastrointestinal complaints. A lack of cortisol also leads to fluid loss, which brings with it the risk of acute renal insufficiency. In addition, there is hyperacidity of the blood, low blood pressure (usually systolic < 100 mm Hg), dizziness, and decreased sodium levels and increased potassium levels in the blood, the latter being associated with an increased risk of cardiac arrhythmias. Cortisol deficiency can further lead to hypoglycemia, which usually takes the form of anxiety, sweating, and palpitations. In addition, psychological disorders, brown skin pigmentation (hyperpigmentation) and the loss of fat and muscle mass can occur. Additional stresses, such as surgery or illness, can cause a sudden worsening of adrenal insufficiency. Especially if still untreated, this leads to a potentially fatal condition characterized by clouding of consciousness to coma, drop in blood pressure, fever, dehydration, and hypoglycemia.
Diagnosis and course
To determine adrenocortical insufficiency, several tests are necessary. In addition to a blood count, 24-hour urine must be collected and a daily cortisol profile must also be established. In addition, it is within the realm of possibility that an ACTH test will be ordered. Because cortisol manages stress, people are especially at risk in stressful situations. The disease can even lead to an Addisonian crisis, which can be fatal. For this reason, a patient must always carry an emergency identification card and cortisone. Addison’s crisis is an extreme drop in cortisol levels. This will initially manifest itself through states of weakness, restlessness and anxiety. But extreme freezing, diarrhea and vomiting will also appear without treatment.However, in this sense, the term stress includes physical exertion, such as surgery, accidents and infectious diseases.
Complications
In the worst case, adrenal insufficiency can lead to the death of the affected person. For this reason, this disease must be treated in any case, since self-healing usually does not occur either. Sufferers of adrenal insufficiency primarily suffer from a general weakness. Furthermore, they lose weight for no particular reason and also suffer from a strong susceptibility to infections. Thus, various infections or inflammations occur more often and more easily. Usually, this disease also leads to severe abdominal pain, vomiting and also nausea. Patients suffer from a strong hunger, preferring especially salty foods. Dizziness or pallor can also occur in adrenal insufficiency and significantly reduce the patient’s quality of life. Furthermore, hypoglycemia and loss of consciousness of the patient may also occur. Adrenocortical insufficiency is treated with the help of medication. There are no particular complications. However, the thyroid gland of the affected person must also be examined, as this can also be affected by a malfunction. With successful and proper treatment, the life expectancy of the affected person is also not reduced.
When should you see a doctor?
Adrenocortical insufficiency usually becomes noticeable only when large parts of the kidney have already been destroyed. Therefore, if possible, the cause should be treated early. People who suffer from alcoholism or diabetes are at risk and must be treated closely to prevent adrenal insufficiency from occurring in the first place. At the latest when signs such as persistent fatigue, nausea and vomiting, and loss of appetite are noticed, the condition must be taken to a doctor. Nonspecific complaints in the gastrointestinal tract also require prompt clarification. In the case of dizzy spells, cardiac arrhythmia or weakness, there may be pronounced dehydration that requires medical treatment. Anxiety and other psychological complaints also indicate a lack of nutrients. Affected individuals are best to speak with their primary care physician, who can diagnose the condition or consult a specialist. Adrenal insufficiency is treated by a nephrologist or other internist. Any psychological complaints that may occur in connection with the condition must be treated therapeutically.
Treatment and therapy
Once the problem of adrenal insufficiency is recognized and therapy is given, there will most likely be no major adverse events if taken properly. Twice-a-day cortisol should usually be taken. However, since some patients experience problems at night, it may be taken several times a day. It is particularly worth knowing that the medication has a long-term effect, which means that high doses are strictly prohibited. In addition, preparations are offered that degrade quickly. These include, for example, hydrocortisone, which is increasingly used in the Addisonian crisis or in emergencies. This can be administered in high doses. Furthermore, adrenal insufficiency patients must not exert themselves too much in everyday life. Every day, the body should be given enough sleep. If there is an infection, then a doctor should be consulted immediately, as the cortisol dose must be increased. After the disease, however, the drug can be slowly dosed down again. If there is secondary hypothyroidism, then thyroid hormones often need to be taken as well. Adrenocortical insufficiency is not curable, but it is easily treatable, which can help maintain quality of life.
Outlook and prognosis
Adrenocortical insufficiency (also known as Addison’s disease) is an incurable condition because once damage to the adrenal cortex occurs, it cannot be reversed. However, if chronic, slow-moving adrenal insufficiency is detected early enough, treatment with vital hormones is possible.This hormone substitution must be taken for a lifetime, but patients can then lead an almost normal life and also have an average life expectancy if the prescribed medication is taken regularly. Very often, pregnancy is even possible in adrenocortical insufficiency if the affected person has their hormone levels checked regularly or if they are well adjusted. Problems can arise if the concentration of hormones does not correspond to the prevailing stress level of the patient. If adrenocortical insufficiency is not treated, the disease is always fatal. There is also a danger to life in the case of an acute Addisonian crisis, especially if it occurs together with the so-called Waterhouse-Friderichsen syndrome. The prospects of a life almost free of symptoms are therefore enormously increased by timely action. Therefore, first symptoms and alarm signs should be taken seriously by those affected in any case.
Prevention
To prevent adrenocortical insufficiency, unfortunately, there are no concrete and proven measures, but you should nevertheless follow some rules. Once you have fallen ill, you should carry an identification card and emergency medication with you. In this way, the situation is always under control. Stress should be avoided for the most part. Before surgery and during infections, the cortisol dose must be increased to avoid getting into an Addisonian crisis. A healthy lifestyle will avoid diseases known to be possible causes of primary and secondary forms of Addison’s disease.
Follow-up
In most cases of adrenocortical insufficiency, very few and often very limited measures of direct follow-up are available to affected individuals. Affected individuals should primarily seek medical attention at a very early stage to prevent and limit the occurrence of other complications and symptoms. Adrenocortical insufficiency usually does not heal on its own, so a doctor should be consulted at the first signs of the disease. As a rule, those affected by adrenal insufficiency are dependent on taking various medications. Those affected should follow the doctor’s instructions and ensure that they take the medication regularly and in the correct dosage. Often, a regular check-up by a doctor is also very important in the case of the disease. Furthermore, the affected person should rest a lot and take it easy, refraining from heavy exertion or physical activities. Likewise, the help and care of one’s family has a positive effect on the further course of the disease and can sometimes prevent the development of psychological upsets.
What you can do yourself
Adrenocortical insufficiency is a hormonal disease. In addition to therapy with cortisone, sufferers can take some steps to improve their well-being. Affected people often feel exhausted. It is therefore advisable to structure the day well. Too many appointments in one day or too many activities in sports or in the household, can lead to overload. Since hypoglycemia often occurs in Addison patients, several small meals at intervals of about 3-4 hours are recommended. In this way, blood sugar remains stable for longer. A banana or a slice of bread in between meals can be enough. Whole grain products are good for blood sugar stability. To prevent hypoglycemia while on the move, it is a good idea to carry dextrose, a sweet drink such as apple juice or a banana in your handbag or backpack. This prevents dangerous hypoglycemia during long walks or appointments. Cortisone tablets and an emergency ID card also belong in the bag. Listening to the body’s signs is also important. If the feeling of exhaustion occurs, a break is advisable, even if others do not understand. Addison’s patients do better when they listen to the body’s signals and learn to be able to say no.