Addison’s disease — Know It All!

Editorial

All you need to know about Hypocortisolism.

Know your ailment well, so you can manage it better!!
Here we come with Addison’s disease today!

Addison’s disease

Overview:

Addison’s disease is also known as Hypocortisolism.

What is Addison’s Disease?

Addison’s disease is a condition in which cortisol and aldosterone receptors are not released sufficiently by the adrenal glands, which rest on top of the kidneys. Cortisol helps the body, including the trauma of sickness, accident, or surgery, adapt to stress.

It also helps to regulate blood pressure, cardiac rhythm, blood glucose (sugar) levels and the immune system. The equilibrium of sodium and potassium in the blood is impaired by aldosterone.

This, in essence, regulates the amount of fluid that the kidneys expel as urine, influencing the volume of blood and blood pressure. “Addison’s syndrome is also referred to as “primary adrenal insufficiency.” A similar condition, “secondary adrenal insufficiency,” occurs when adequate adrenocorticotropic hormone (ACTH) is not secreted by the pituitary, a small gland at the base of the brain, which stimulates the adrenal glands to generate cortisol.

How Common is Addison’s disease in autoimmune disorders?

About 70 % of reported cases of Addison’s disease are due to autoimmune disorders, in which the immune system makes antibodies that attack the body’s own tissues or organs and slowly destroy them.

Addison’s disease

What causes Addison’s disease?

Addison’s disease is caused by damage to the adrenal glands, resulting in inadequate cortisol hormones and also, insufficient aldosterone. You are part of the endocrine system with the adrenal glands. They create hormones that provide direction to almost every organ and tissue in your body.

Two pieces are made of the adrenal glands. The interior (medulla) contains hormones resembling adrenaline. A group of hormones called corticosteroids are formed by the outer layer (cortex). Among the corticosteroids are:

With glucocorticoids. These hormones, which include cortisol, affect the capacity of your body to turn food into energy, play a role in the inflammatory reaction of your immune system and enable your body to respond to stress.

About mineralocorticoids. These hormones, which contain aldosterone, regulate the sodium and potassium balance in the body in order to keep blood pressure normal.

About androgens. In both males and women, these male sex hormones are released in limited quantities by the adrenal glands. In both men and women, they induce sexual growth in men and affect muscle mass, sex desire (libido) and a sense of well-being.

Primary adrenal insufficiency

The disorder is termed primary adrenal insufficiency when the cortex is weakened and doesn’t contain enough adrenocortical hormones. Most frequently, this is the result of the body fighting itself (an autoimmune disease). Your immune system sees the adrenal cortex as alien, something to strike and kill for unexplained reasons. People with Addison’s disorder are much more prone to have another autoimmune condition than some.

Other reasons for the failure of the adrenal glands can include:

With tuberculosis

Some adrenal gland diseases

Diffusion of tumours of the adrenal glands

Into the adrenal glands to bleed. In this scenario, without any prior signs, you might have an Addisonian problem.

In this scenario, without any prior signs, you might have an Addisonian problem.

Secondary adrenal insufficiency

A hormone called adrenocorticotropic hormone is released by the pituitary gland (ACTH). In exchange, ACTH activates the adrenal cortex to develop the hormones. Inflammation, benign pituitary tumours and previous pituitary surgery are common causes of inadequate pituitary hormone development.

Too little ACTH will lead to too little of the glucocorticoids and androgens your adrenal glands usually produce, even if they do not affect your adrenal glands themselves. This is considered adrenal secondary insufficiency. The development of mineralocorticoids is not impacted by too little ACTH.

The bulk of secondary adrenal insufficiency symptoms are close to primary adrenal insufficiency symptoms. However, there is no hyperpigmentation in individuals with secondary adrenal insufficiency and they are less likely to experience extreme vomiting or low blood pressure. It’s more likely because they have low blood sugar.

For people who use corticosteroids (for example, prednisone) to treat chronic disorders, such as asthma or arthritis, avoid taking the corticosteroids all at once rather than tapering down, a transient source of secondary adrenal insufficiency emerges.

Addison’s disease

What are the symptoms of Addison’s Disease?

People who have Addison’s disease may experience the following symptoms:

  • muscle weakness
  • fatigue and tiredness
  • darkening in skin colour
  • weight loss or decreased appetite
  • a decrease in heart rate or blood pressure
  • low blood sugar levels
  • fainting spells
  • sores in the mouth
  • cravings for salt
  • nausea
  • vomiting

People living with Addison’s disease may also experience neuropsychiatric symptoms, such as:

  • irritability or depression
  • lack of energy
  • sleep disturbances

If Addison’s disease goes untreated for too long, it can become an Addisonian crisis. Symptoms associated with an Addisonian crisis can include

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  • agitation
  • delirium
  • visual and auditory hallucinations

An Addisonian crisis is a life-threatening medical emergency. Call 911 immediately if you or someone you know begins to experience:

  • mental status changes, such as confusion, fear, or restlessness
  • loss of consciousness
  • high fever
  • sudden pain in the lower back, belly, or legs

An untreated Addisonian crisis can lead to shock and death.

Addison’s disease

What are the complications of Addison’s disease:

  • You can experience an Addisonian crisis as a result of physical stress, such as accident, illness or disease, whether you have untreated Addison’s disease. Normally, in response to physical stimulation, the adrenal glands release two to three times the normal amount of cortisol. With adrenal insufficiency, the failure to increase the output of cortisol with stress can lead to an additional crisis.
  • A life-threatening situation that occurs in low blood pressure, low blood sugar and high blood potassium levels is a life-threatening problem. Immediate medical attention would be required for you.
  • People with Addison’s disease usually have autoimmune disorders associated with it.
Addison’s disease

How to diagnose Addison’s Disease? :

To determine if you have Addison’s disease, your doctor might use the following:

  • History and physical: Your doctor will review your symptoms and perform a physical exam. Dark patches on your skin might be a clue for your doctor to consider testing for Addison’s disease.
  • Blood tests: These will be done to measure the levels of sodium, potassium, cortisol and ACTH in your blood.
  • ACTH stimulation test: This tests the adrenal glands’ response after you are given a shot of artificial ACTH. If the adrenal glands produce low levels of cortisol after the shot, they may not be functioning properly.
  • X-rays: These may be done to look for calcium deposits on the adrenal glands.
  • Computed tomography (CT scan): Computed tomography uses computers to combine many X-ray images into cross-sectional views. A CT scan might be done to evaluate the adrenals and/or pituitary gland. For example, it can show if the immune system has damaged the adrenal glands or if the glands are infected.

How to Treat Addison’s disease :

Medication is used in all care with Addison’s disease. In order to fix the levels of steroid hormones the body does not generate, you will be offered hormone replacement therapy. Oral corticosteroids have certain choices for therapy, such as:

Hydrocortisone (Cortef), prednisone, or cortisol-replacement methylprednisolone. To simulate the usual 24-hour fluctuation of cortisol levels, these hormones are given on a schedule.

  • Fludrocortisone acetate for aldosterone replacement.
  • Particularly after physical exercise, when the weather is hot or if you have bowel upsets, such as diarrhoea, you will need to get lots of salt (sodium) in your diet.
  • When your body is exhausted, such as after a procedure, an infection or a mild disease, your doctor will sometimes prescribe a temporary rise in your drug dosage. You will require shots of corticosteroids if you are sick with dehydration and are unable to hold oral medicine down.

Medical Care:

  • IV access should be urgently identified in patients in acute adrenal crisis, and isotonic sodium chloride solution infusion should be initiated to recover volume deficiency and correct hypotension. Glucose supplementation may be needed in some patients. Where appropriate, the precipitating trigger should be found and corrected.
  • The typical adrenal gland release of cortisol is approximately 250–300 mg in 24 hours in stress situations. This volume of soluble hydrocortisone (sodium succinate hydrocortisone or phosphate) should be prescribed, preferably through a continuous infusion.
  • Administer 100 mg of hydrocortisone by continuous IV infusion at a rate of 10–12 cc/h in 100 cc of isotonic sodium chloride solution. An injection of 100 mg of hydrocortisone as an IV bolus can be initiated. In certain clinics, 300–400 mg is blended in 1 litre of saline and injected over 24 hours to prevent the need to restart infusion after 8–10 hours.
  • Per 6–8 hours, 100 mg as an IV bolus is an alternative form of hydrocortisone administration.
  • The method of infusion holds plasma cortisol levels more adequately at levels of steady stress especially in the small percentage of patients who are fast metabolizers and may have low levels of plasma cortisol between IV boluses.
  • Within 4–6 hours of hydrocortisone infusion, clinical improvement, especially blood pressure reaction, should be apparent. Otherwise, that would be a doubtful diagnosis of adrenal insufficiency.
  • The dosage of tension hydrocortisone should be lowered to 100–150 mg after 2–3 days and injected over a 24-hour cycle, regardless of the health state of the patient. Which is to prevent gastrointestinal bleeding from tension.
  • If the patient progresses and if the clinical condition requires, the injection of hydrocortisone can be progressively lowered to daily replacement doses of approximately 3 mg/h (72–75 mg over 24 h) over the next 4–5 days and finally to daily oral replacement doses if oral consumption is necessary.
  • No mineralocorticoid replacement is required before the patient receives 100 mg or more of hydrocortisone within 24 hours. At this dosage, the mineralocorticoid activity of hydrocortisone is adequate.
  • After that when the hydrocortisone dosage is more weaned, mineralocorticoid substitution at doses equal to the normal adrenal gland aldosterone production of 0.05–0.20 mg per 24 hours should be added. 9-alpha-fludrocortisone, typically in doses of 0.05–0.10 mg every day or any other day, is the normal mineralocorticoid used for this reason.
  • It could be important to encourage patients to increase their salt consumption in hot weather.

Surgical Care:

In periods of major tension, trauma, or surgery and for every major operation, parenteral steroid coverage should be used.

During surgical operations, 100 mg of hydrocortisone should be given prior to the start of a continuous IV infusion, preferably by the IM route. In the event of issues with IV entry, an IM dosage of hydrocortisone guarantees steroid coverage.

An intermittent IV bolus injection every 6–8 hours can be used where continuous IV infusion is not feasible.

Hydrocortisone should be easily tapered to the normal replacement doses within 24–36 hours of the treatment, or as progressively as the clinical condition requires.

Mineralocorticoid replacement will normally be delayed before daily replacement steroids are restored by the patient

Prevention :

  • The epidemic of Addison can not be avoided, but there are steps you can take to stop the tragedy of Addison:
  • If you are still feeling exhausted, sluggish, or are losing weight, talk to the doctor. Ask if you have a lack of adrenals.
  • When you have been diagnosed with Addison’s disease, ask the doctor if you are ill and see what to do. You may need to learn how to raise the corticosteroid dosage.
  • Go to the emergency room if you get really ill, particularly if you are vomiting and you are unable to take your medicine.
  • Some individuals with Addison’s disease are worried about the adverse side effects of hydrocortisone or prednisone and they are mindful that these exist for other causes in people who take these steroids.
  • However, the side effects of high-dose glucocorticoids do not exist if you have Addison’s disease, since the dose you are given eliminates the quantity that is absent. Be sure to check up on a daily basis with your doctor to make sure that your dosage is not too high.

 

References:

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https://www.mayoclinic.org/diseases-conditions/addisons-disease/diagnosis-treatment/drc-20350296

https://www.healthline.com/health/why-you-should-avoid-adrenal-extract

https://my.clevelandclinic.org/health/diseases/15095-addisons-disease/management-and-treatment

https://www.msdmanuals.com/home/hormonal-and-metabolic-disorders/adrenal-gland-disorders/addison-disease#v772490

https://emedicine.medscape.com/article/116467-treatment#d8