What Is Addison’s Disease?

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Are you experiencing bouts of fatigue or extreme weakness? These symptoms can be unpleasant to experience and they are usually associated with common health conditions, such as the flu. But sometimes they could be a sign of a rare health condition called Addison’s disease, especially when these symptoms occur daily. Diagnosing Addison’s disease can be tricky and it is hard to spot early on. If Addison’s disease is left untreated then an adrenal crisis can occur. It can be hard living with this disease, but there is medication that is taken daily to help people live a healthy life.

This article will take a closer look into Addison’s disease, what can cause it, the different symptoms associated with the condition, and the different treatment options available. This article will help you to gain a better understanding surrounding Addison’s disease and adrenal crisis. 

Overview

Addison’s disease (named after Dr Thomas Addison) is a rare condition where the adrenal glands do not function properly. Therefore, they do not produce enough adrenal hormones called cortisol and aldosterone, which are important for our body. The adrenal glands are two small glands that are located on top of your kidneys. Their job is to produce cortisol and aldosterone so our body functions properly. Cortisol is a hormone that helps our body to respond to stress, but it also helps to control our blood pressure, blood sugar, and metabolism. Aldosterone controls the salt and water balance in our bodies, as well as blood pressure. From this information, you can see how vital adrenal function is to keep our body working in a healthy way.

Around 9000 people are affected by Addison’s disease in the UK, so this condition is rare. People tend to develop the condition between the ages of 30 and 50 and it is seen more commonly in people assigned female at birth than in people assigned male at birth. If Addison’s disease is left untreated, an adrenal crisis can occur, which is fatal if not treated.1

Addison’s disease is also known as primary adrenal insufficiency.1 Adrenal insufficiency means that the adrenal glands are not working properly and hence, are not able to effectively produce adrenal hormones. There are different reasons why adrenal insufficiency can occur and they are split into 3 main types: primary adrenal insufficiency, secondary adrenal insufficiency, and tertiary adrenal insufficiency. Primary adrenal insufficiency occurs as a result of the adrenal glands being directly damaged. Secondary adrenal insufficiency can occur when the pituitary gland, a structure located at the base of our brain, stops releasing adrenocorticotropic hormone (ACTH), which is needed to send signals to the adrenal glands to produce the adrenal hormones. Tertiary adrenal insufficiency occurs when the hypothalamus, a small area of the brain, does not work properly. Due to this, the hypothalamus can no longer send hormonal signals to the pituitary gland and subsequently, no signal reaches the adrenal glands to produce adrenal hormones.

Causes of addison's disease

There are different reasons as to why Addison’s disease may develop. Usually, Addison’s disease occurs due to an autoimmune disease.1 This is when the body’s immune system mistakenly attacks your body’s own cells, such as the cells of the adrenal gland. Addison’s disease can also be caused by an infection called Tuberculosis. It usually affects the lungs but it can spread to other parts of your body, such as your adrenal glands, and cause damage. Yet, there are also less common causes of Addison’s disease, including cancer spreading to your adrenal gland, bleeding into the adrenal gland, and surgical removal of the adrenal gland due to another health condition.1 Genetic disorders can also cause Addison’s disease. An example is congenital adrenal hyperplasia, where a mistake in the genetic code causes less cortisol to be produced.  

Signs and symptoms of addison’s disease

It is hard to detect Addison’s disease early because many of the initial mild symptoms are seen in other health conditions. The symptoms experienced can be different for everyone and they gradually worsen over time. Because of this, Addison’s disease is usually diagnosed at a later stage, when more severe symptoms are present.

The common symptoms of Addison’s disease are:1

  • Fatigue or exhaustion
  • Muscle weakness
  • Weight loss
  • Loss of appetite
  • Hyperpigmentation (darker patches of skin)
  • Gastrointestinal issues, such as vomiting, nausea, diarrhoea, or abdominal pain
  • Hypotension (low blood pressure)

There are other symptoms that are less commonly seen. Some people may experience vitiligo, which is when white patches appear on your skin.2 Other symptoms can include muscle pain, or spasms, and light-headedness.2 Another symptom of Addison’s disease is cravings for salty foods.Some people may experience low blood sugar levels as well. Females may see irregular periods or they may stop menstruating altogether. Due to this, they may have a decreased sexual drive. It is important to see a doctor if you are worried about any of the symptoms above.

Even though Addison’s disease usually develops gradually, sometimes symptoms can appear suddenly. This is known as acute adrenal failure or adrenal crisis and this is a life-threatening condition. An adrenal crisis can be triggered by a significant, stressful situation, such as an illness, infection, or an operation.

The symptoms of an adrenal crisis are severe and include:3

  • Hypotension (low blood pressure)
  • Severe dehydration
  • Abdominal pain and tenderness
  • Nausea and vomiting
  • Dizziness
  • Fever
  • Severe weakness
  • Loss of consciousness

Health professionals, such as doctors, can help to diagnose Addison’s disease. Your doctor will ask about your symptoms and have a look at your medical history. They may also have a closer look at your skin to see if you have any hyperpigmentation, which are dark patches on your skin, or vitiligo, which are white patches on your skin. These symptoms do not occur in everyone with Addison’s disease.  

You may also have a blood test done to examine the levels of sodium, potassium, and cortisol in your body. A low sodium, high potassium or low cortisol level could be an indicator of Addison's disease. An ACTH stimulation test can also be used to measure the hormone level of cortisol in your blood.1 ACTH is the adrenocorticotropic hormone that signals to the adrenal glands to make cortisol. If, after the test, the cortisol level is low, then this may suggest Addison’s disease. Imaging tests such as a computed tomography (CT) scan can help to check the size of your adrenal glands and to detect other symptoms of Addison’s disease.1

If other diagnostic tests don’t give a firm diagnosis, then an Insulin-induced hypoglycemia test is performed. This helps to distinguish between Addison’s disease and secondary adrenal insufficiency, which is caused by the pituitary gland not working properly.

Management and treatment for addison’s disease

If you are diagnosed with Addison’s disease, then you will need to take medication every day to help replace the hormones that your adrenal glands aren't producing. This will help to make sure your body is functioning properly. A corticosteroid is given to replace the cortisol, with the most common medicine given being hydrocortisone.1,4 Less commonly doctors, may prescribe other medication, such as prednisolone or dexamethasone, to replace the cortisol.1,4 Fludrocortisone can also be given to replace the aldosterone that is underproduced.1,4 In some cases, people may also take dehydroepiandrosterone (DHEA) to improve their sexual drive.1

The doctor will adjust the dose of each medication to your needs. It is important to take your medication on time because missing a dose of your medicine, or taking it late, can cause fatigue. It can also help to avoid an adrenal crisis, which is a serious health condition.

Other key recommendations include:

  • Keeping spare medication with you, for example, in your car, at work, or when you travel
  •  Remembering to order and collect your repeat prescriptions on time
  • Packing extra medication when going on holiday
  • Remembering to take your medication at the right times each day
  • Carrying around your steroid emergency card so that healthcare professionals are aware of how to help you quickly in an emergency 
  • Keeping a hydrocortisone injection kit with you, which is only used in emergencies

Medication will need to be adjusted in periods of physical stress, such as if you are ill, experiencing emotional stress, or having any operations.1 Your doctor will advise you on the dosage to take.

If someone is having an adrenal crisis, it is considered as a medical emergency and the person needs to be treated urgently. A hydrocortisone injection is given to the person immediately. When in a hospital, the person will receive fluids containing salts and sugar to help with rehydration. They will have further treatment with hydrocortisone.3

FAQs

How is Addison’s disease diagnosed?

The doctor will assess your signs and symptoms and check your medical records. Symptoms can include weakness, fatigue, and low blood pressure.1 They may have a look at your skin to see if you have hyperpigmentation or vitiligo, which can develop if someone has Addison’s disease.1 Blood tests can be done to check the levels of sodium, potassium, and cortisol, because abnormalities in the levels of these elements could suggest Addison’s disease. Other diagnostic tools include an ACTH stimulation test or a CT scan.

Can I prevent addison’s disease?

Unfortunately, Addison’s disease cannot be prevented. 

Who isat risk of addison’s disease?

You may be at risk of Addison’s disease if you have an autoimmune disease, cancer or an infection (such as tuberculosis). It can also occur if your adrenal glands have been removed during surgery or if you have a genetic disorder that predisposes you to Addison’s disease.

How common is addison’s disease?

According to the NHS, there are around 9000 people with Addison’s disease in the UK. It is a rare disease which is more common in people assigned female at birth than in people assigned male at birth.

When should I see a doctor?

You should see a doctor if you exhibit any signs or symptoms of Addison’s disease that are discussed in this article or any other signs that concern you. If you have an adrenal crisis, this is a medical emergency and you must receive medical care as soon as possible.

Summary

Addison’s disease is a rare, life-long health condition where the adrenal glands don’t work properly. This means that the vital hormones, cortisol and aldosterone, aren’t produced, so the body does not function properly. The most common cause of Addison’s disease are autoimmune diseases that damage the adrenal glands, but there are other causes, such as tuberculosis. Seeing as symptoms gradually develop and can overlap with other common health conditions, it is hard to diagnose Addison’s disease early on. If Addison’s disease is left untreated, then an adrenal crisis can occur, which is a medical emergency. Addison’s disease can be treated with medication which helps to replace the hormones that are underproduced. The medication helps with the symptoms and allows people to lead a normal life. If you have any concerns or symptoms relating to Addison’s disease, then it is important to speak to your doctor.

References

  1. Michels A, Michels N. Addison disease: early detection and treatment principles. afp [Internet]. 2014 Apr 1 [cited 2023 Feb 10];89(7):563–8. Available from: https://www.aafp.org/pubs/afp/issues/2014/0401/p563.html
  2. Choudhary S, Alam A, Dewan V, Yadav D, Dubey NK. An Unusual Presentation of Addison’s Disease—A Case Report. Clinical Pediatric Endocrinology [Internet]. 2011 Jul 1 [cited 2020 Mar 26];20(3):57–60. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3687640/ 
  3. Dineen R, Thompson CJ, Sherlock M. Adrenal crisis: prevention and management in adult patients. Therapeutic Advances in Endocrinology [Internet]. 2019 Jan [cited 2023 Feb 10];10:204201881984821. Available from: http://journals.sagepub.com/doi/10.1177/2042018819848218 1.
  4. Bornstein SR, Allolio B, Arlt W, Barthel A, Don-Wauchope A, Hammer GD, et al. Diagnosis and treatment of primary adrenal insufficiency: an endocrine society clinical practice guideline. The Journal of Clinical Endocrinology & Metabolism [Internet]. 2016 Feb [cited 2023 Feb 10];101(2):364–89. Available from: https://academic.oup.com/jcem/article-lookup/doi/10.1210/jc.2015-1710 

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This content is purely informational and isn’t medical guidance. It shouldn’t replace professional medical counsel. Always consult your physician regarding treatment risks and benefits. See our editorial standards for more details.

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Antanina Sivirentsava

Master of Pharmacy, MPharm - University of East Anglia, Norwich

Antonia is a recent pharmacy graduate who is passionate about communicating complex scientific information in an easy and accessible way to improve the general public’s wellbeing and quality of life. She has a strong interest in medical communications and has aspirations of working as a medical writer.

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