The Adrenal glands are the producer of hormones, which sits atop of your kidneys. The outer part of the gland is called the Adrenal cortex, which produces the hormones Cortisol and Aldosterone. When the cortex is not producing the normal hormone level it is known as Adrenal Insufficiency. According to the Mayo clinic, Adrenal Insufficiency is a disorder that is caused when Adrenal glands produce too little Cortisol and often Aldosterone. This condition affects both men and women and can happen at any age. When your adrenal glands are not functioning properly your entire body could be affected. As cortisol plays a role in converting your food into energy keeping your immune system inflammatory response in check and helps your body respond to stress. Aldosterone helps to keep your blood pressure normal. Symptoms of adrenal insufficiency include extreme fatigue, nausea or vomiting, depression, and darling of your skin among others. The failure of your adrenal glands to produce a sufficient amount of hormones is most commonly the result of an autoimmune disease, but also can be caused by tuberculosis, the spread of cancer to the adrenal glands, and infection of the adrenal glands among others. Treatments are available that can help correct or replace hormones in your system, so it’s important to speak to your doctors if you have any of these symptoms. The term adrenal insufficiency refers to as a syndrome, while by the specific diseases that cause a syndrome are differentiated as the primary adrenal insufficiency referring to the adrenal glands as the source of the pathology or the secondary adrenal insufficiency referring to the hypothermic or the pituitary as the source of the pathology.
PRIMARY ADRENAL INSUFFICIENCY
This is also known as Addison's Disease is a rare endocrine disorder that happens when the adrenal gland isn’t able to produce enough of the hormones that the body needs particularly aldosterone and cortisol. The reason it is called as primary is that the underlying problems localized to the adrenal glands itself. Rather than a problem of a hormone that acts on the adrenal gland or elsewhere in the body. We need to first understand the adrenal gland and its functions. The inner part of the adrenal gland is called the Medulla, this Medulla secretes the catecholamine. The outer part is the Cortex, this secretes aldosterone, cortisol, and sex steroids. Let us first understand the functions of these hormones
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ALDOSTERONE
Aldosterone increases sodium and water reabsorption in the kidneys.
It also causes excretion of potassium in the kidneys.
It also helps to maintain normal blood pressure
CORTISOL
Cortisol stimulates gluconeogenesis in the liver and helps to maintain normal glucose concentration of the blood
It also stimulates lipolysis throughout the body
It depresses the immune response
It also helps to decrease the inflammation
SEX STEROIDS
They are mainly associated with the development of secondary sexual characteristics.
The deficiency in the secretion of all these hormones which causes to develop Addison's disease If we see the etiology of Addison's disease, if we see the causes for this it would include.
CAUSES
AUTOIMMUNE ADRENALITIS
In this case, the bodies own B cells and the D cells attack the adrenal glands and damage it.
INFECTIONS
This is caused by organisms like mycobacterium, tuberculosis, and also fungal infections like Histoplasmosis.
ADRENAL INFECTION
This happens in cases of systemic lupus erythematosus and antiphospholipid antibody syndrome.
DRUGS
Drugs like mitotic and ketoconazole, when taken in excess, can damage the adrenal glands.
METASTASIS
Metastasis from the organs like lungs and the stomach can also damage the adrenal glands.
TRAUMA
Trauma to the abdomen either blunt or sharp will also cause damage to the adrenal gland.
ADRENAL HEMORRHAGE
This happens in the case of Waterhouse Fredrickson syndrome and anticoagulation therapy can also cause adrenal insufficiency.
STORAGE DISORDERS
Storage disorders like amyloidosis and hemochromatosis also affect the adrenal gland.
SURGERY
The surgical removal of the adrenal gland which is also known as the bilateral adrenalectomy causes Addison's disease.
CLINICAL FEATURES
CORTISOL DEFICIENCY
The clinical features due to cortisol deficiency usually manifest as fatigue, anorexia, weight loss, muscle weakness, abdominal pain, diarrhea, and hypoglycemia which manifests as sweating and headache. Another symptom is the hyperpigmentation which occurs in areas like hands and the buccal mucosa. It is important to understand why the hyperpigmentation occurs, the pituitary secretes Melanocyte-stimulating Hormone (MSH) and Adrenocorticotropic Hormone (ACTH). The ACTH acts on the adrenal and causes an increase in the secretion of cortisol and rest is converted into alpha MSH. In the case of Addison's disease, the cortisol secretion is decreased through a feedback machinist, there is a reflex that causes the increase of the secretion of MSH and ACTH. The increase in MSH causes hyperpigmentation.
ALDOSTERONE DEFICIENCY
Since aldosterone is important to maintain normal blood pressure, it’s deficiency manifests as low blood pressure. These patients also have orthostatic hypotension, as the blood pressure further falls when they rise up after sitting. In this, the patents have a craving for salty food.
SEX STEROID DEFICIENCY
This manifests as a loss of libido in both sexes and menstrual irregularities in females.
DIAGNOSIS
Blood glucose measurement shows a low plasma blood group.
Plasma cortisol between 8 am to 9 am shows a value of fewer than 3 mg/DL.
ACTH stimulation test is important to diagnose adhesions disease.
Plasma ACTH levels are usually elevated 100<gm/DL.
Autoimmune adrenalitis organ-specific antibodies can be deducted like adrenal cortex antibodies 21 hydroxylase antibodies.
SECONDARY ADRENAL INSUFFICIENCY
This is also called acute secondary Adrenal insufficiency, meaning the pathology has been present for days or weeks or chronic, meaning the pathology has been present for weeks or months. The pathology of the level of the hypothalamus or the pituitary. With the pathology at the level of the pituitary such that the (Adrenocorticotropic hormone) production is deficient since cortisol is entirely dependent upon ACTH, even though are healthy and capable there is no cortisol synthesis. Thus the pathognomonic laboratory characteristics include low basal cortisol, inappropriately low ACTH. Some patients might be hyponatremic, however, since aldosterone regulations are not dependent on ACTH potassium homeostasis should be unimpaired. The clinical manifestation in secondary Adrenal insufficiency depends upon the degree of stress the patience is facing and the relative deficiency of cortisol in that degree of stress. Thus the manifestation can range from almost no abnormalities to mild, moderate, or severe signs of fatigue, orthostasis, hypotension, in the situations of stress. Since the adrenal glands in acute secondary Adrenal insufficiency are intact, in response to exogenous cosyntropin they are expected to display a robust cortisol secretion. However, if the deficiency of ACTH should continue for weeks or months over time it will lead to decreased secretion of adrenal androgens and cortisol.
CAUSES
Secondary Adrenal insufficiency can be caused by an insult or injury to the hypothalamus of the pituitary which can include
Pituitary inflated
Inflated tumors
Infections of the pituitary (granulomatosis disease, iron)
Radiation
The most common cause is the medication, which includes
Glucocorticoids can bind to the hypothalamic and pituitary glucocorticoid receptor and suppress the entire hypothalamic pituitary adrenal access.
Opioids
A condition that damages your pituitary gland will eventually result in reduced production of ACTH, this then further leading to the cortex not receiving sufficient hormones to stimulate it.
A sudden miss of corticosteroid drugs that are used to treat conditions like Asthma or Arthritis will also lead to Cortex malfunction and which is also known as Secondary Adrenal Insufficiency
This state would result in a reduction in the secretion of Cortisol and Aldosterone, even though there is no issue in the adrenal cortex.
TREATMENT
In this case, the patients require glucocorticoid replacement such as prednisone or hydrocortisone.
FAQs on Adrenal Insufficiency
1. What is Adrenal Insufficiency?
Adrenal Insufficiency is a medical condition where the adrenal glands, located on top of the kidneys, do not produce enough of certain crucial hormones. The primary hormones affected are cortisol, which helps the body respond to stress, and often aldosterone, which regulates blood pressure and the body's salt and water balance.
2. What are the common symptoms of Adrenal Insufficiency?
The symptoms of Adrenal Insufficiency often develop gradually and can include:
- Chronic, worsening fatigue and muscle weakness
- Unintentional weight loss and decreased appetite
- Low blood pressure (hypotension), which can cause dizziness upon standing
- Darkening of the skin (hyperpigmentation), especially in skin creases, scars, and gums
- Salt cravings
- Nausea, vomiting, or abdominal pain
- Low blood sugar (hypoglycemia)
3. How is Adrenal Insufficiency diagnosed?
Diagnosing Adrenal Insufficiency involves a combination of a physical examination and specific tests. A doctor may order:
- Blood tests: To measure the levels of sodium, potassium, cortisol, and Adrenocorticotropic hormone (ACTH) in your blood.
- ACTH Stimulation Test: This is the most definitive test. It measures the level of cortisol in your blood before and after an injection of synthetic ACTH. If the adrenal glands are damaged, they won't produce much cortisol in response to the ACTH signal.
- Imaging tests: A CT scan or MRI of the abdomen or pituitary gland may be used to check for physical damage or tumours.
4. What is the standard treatment for Adrenal Insufficiency?
Treatment for Adrenal Insufficiency involves hormone replacement therapy to replace the hormones your body is no longer making. This typically includes daily oral corticosteroids, such as hydrocortisone or prednisone to replace cortisol, and sometimes fludrocortisone acetate to replace aldosterone. The dosage is carefully managed to mimic the body's natural rhythm and must be increased during times of physical stress.
5. What happens during an adrenal crisis?
An adrenal crisis, or acute adrenal insufficiency, is a life-threatening medical emergency. It occurs when the cortisol levels in the body drop suddenly. It can be triggered by physical stress like an injury, surgery, or severe infection. Symptoms are sudden and severe, including:
- Sudden, penetrating pain in the lower back, abdomen, or legs
- Severe vomiting and diarrhoea, leading to dehydration
- Loss of consciousness or confusion
- A sharp drop in blood pressure
- High fever
This condition requires immediate medical attention and an emergency injection of hydrocortisone.
6. What is the difference between primary and secondary Adrenal Insufficiency?
The key difference lies in the root cause of the hormone deficiency. Primary Adrenal Insufficiency (Addison's disease) occurs when the adrenal glands themselves are damaged and cannot produce enough cortisol. In contrast, Secondary Adrenal Insufficiency begins with a problem in the pituitary gland, which fails to produce enough ACTH. Without ACTH to signal them, the healthy adrenal glands do not produce cortisol.
7. Is 'adrenal fatigue' the same as Adrenal Insufficiency?
No, they are not the same. Adrenal Insufficiency is a recognised and diagnosable medical condition confirmed by blood tests, which requires lifelong hormone replacement therapy. 'Adrenal fatigue' is a non-medical term used to describe a collection of non-specific symptoms like fatigue and body aches. It is not a recognised medical diagnosis, and its symptoms can often be linked to other underlying health issues or lifestyle factors.
8. How do deficiencies in cortisol and aldosterone cause the specific symptoms of Adrenal Insufficiency?
Each hormone deficiency contributes to specific symptoms. A lack of cortisol disrupts the body's ability to manage stress, maintain blood sugar, and control inflammation, leading to fatigue, weight loss, and hypoglycemia. A lack of aldosterone disrupts the body's salt and water balance. This causes the kidneys to excrete too much sodium and retain too much potassium, leading to low blood pressure, salt cravings, and dizziness.
9. What is a 'stress dose' and why is it necessary for patients with Adrenal Insufficiency?
A 'stress dose' is an increased dose of corticosteroid medication taken during times of physical stress, such as fever, infection, surgery, or serious injury. It is necessary because a healthy person's adrenal glands would naturally produce a surge of extra cortisol to cope with such stress. Since a person with Adrenal Insufficiency cannot do this, they must manually increase their medication to mimic this natural response and prevent a life-threatening adrenal crisis.
10. Are the symptoms of Adrenal Insufficiency different in males and females?
The core symptoms of Adrenal Insufficiency, such as fatigue, low blood pressure, and weight loss, are generally the same for both males and females. However, since the adrenal glands also produce small amounts of sex hormones (androgens), females with the condition may experience additional symptoms like loss of body hair (especially pubic and underarm hair) and a decreased sex drive. These effects are less noticeable in males, as their testes produce the vast majority of their androgens.
