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Addison's Disease

What is Addison's Disease?

Addison´s disease is a rare endocrine or hormonal disorder characterized by a diminished production of hormones by the adrenal glands, two small organs located above each kidney. The adrenal glands have two parts: an inner part or medulla that produces adrenaline and an outer part or cortex that produces cortisol and aldosterone. In Addison´s disease these hormones are absent or present at very low levels. Adrenaline is produced upon a “dangerous situation” and prepares the body to escape from it (increasing heart rate and levels of sugar in the blood).  Its absence does not cause any symptoms. However, the hormones cortisol and aldosterone are involved in the fine tuning of critical process of the body. Cortisol controls how the food that we eat is used as fuel, slows the inflammation response of the immune system, and helps control the amount of water in the body. Aldosterone regulates the levels of salt and water in the blood, what ultimately afects the blood volume and blood pressure. The lack of these two hormones can cause fatigue, abnormalities of the gastrointestinal gut (vomiting, diarrhea), weight loss, darkening of the skin, and mood changes among others. The symptoms usually develop slowly but sometimes they can escalate quickly in what is known as an addisonian crisis or acute adrenal failure. This is a medical emergency that needs to be treated inmediately.

Addison´s disease can be caused by the destruction of the adrenal glands (classic Addison´s disease or primary adrenal insufficiency) or by the inability to generate the signals that estimulate the adrenal glands to produce these hormones (secondary adrenal insufficiency).

Addison´s disease occurs in all age groups and it affects men and women equally.

 

Synonyms

  • Primary Adrenal Insufficiency
  • Chronic adrenocortical insufficiency
  • Primary failure adrenocortical insufficiency

Addison´s disease is a rare endocrine or hormonal disorder characterized by a diminished production of hormones by the adrenal glands, two small organs located above each kidney. The adrenal glands have two parts: an inner part or medulla that produces adrenaline and an outer part or cortex that produces cortisol and aldosterone. In Addison´s disease these hormones are absent or present at very low levels. Adrenaline is produced upon a “dangerous situation” and prepares the body to escape from it (increasing heart rate and levels of sugar in the blood).  Its absence does not cause any symptoms. However, the hormones cortisol and aldosterone are involved in the fine tuning of critical process of the body. Cortisol controls how the food that we eat is used as fuel, slows the inflammation response of the immune system, and helps control the amount of water in the body. Aldosterone regulates the levels of salt and water in the blood, what ultimately afects the blood volume and blood pressure. The lack of these two hormones can cause fatigue, abnormalities of the gastrointestinal gut (vomiting, diarrhea), weight loss, darkening of the skin, and mood changes among others. The symptoms usually develop slowly but sometimes they can escalate quickly in what is known as an addisonian crisis or acute adrenal failure. This is a medical emergency that needs to be treated inmediately.

Addison´s disease can be caused by the destruction of the adrenal glands (classic Addison´s disease or primary adrenal insufficiency) or by the inability to generate the signals that estimulate the adrenal glands to produce these hormones (secondary adrenal insufficiency).

Addison´s disease occurs in all age groups and it affects men and women equally.

Acknowledgement of Addison's Disease has not been added yet.

The prevalence of Addison´s disease is estimated to be 1 in 100,000. Prevalence is the proportion of the population that suffer from a condition in a given period of time.

Name Abbreviation
Primary Adrenal Insufficiency Addison's disease
Chronic adrenocortical insufficiency Addison's disease
Primary failure adrenocortical insufficiency Addison's disease

Primary adrenal insufficiency or classic Addison´s disease is caused by the loss of the adrenal glands function. The most common cause is the destruction of the adrenal glands by an autoimmune reaction. The natural defenses of the body wrongfully detect the adrenal glands as an external threat and destroy them. It is estimated that 90% of the adrenal glands should be destroyed in order for the symptoms to appear.

Tuberculosis used to be the most common cause of Addison´s disease in the past, and it is still the case in developing countries.

Other more rare causes include the metastasis of cancer cells to the adrenal glands, chronic infections (mainly fungal), bleeding in the adrenal glands due to a trauma, amyloidosis (the accumulation of amyloid protein in the adrenal glands) or surgical removal of the glands.

Secondary adrenal insufficiency is caused by the lack of another hormone called ACTH or adrenocoticotropic hormone. ACTH is secreted by the pituitary gland in the brain and serves to indicate the adrenal gland that cortisol needs to be produced. In this case, aldosterone is present. Secondary adrenal insufficency is considered a different medical entity than Addison´s disease.

The symptoms of Addison´s disease usually begin gradually and may worsen over time. The most characteristic symptoms are fatigue, unintended weight loss and loss of appetite. Low blood pressure is also common, and can cause lightheadedness when standing and even fainting sometimes. It is characteristic the darkening of the skin (hyperpigmentation) in exposed and unexposed areas. Weakness of the muscles can also happen, and more rarely the muscles can suffer spasms (uncontrolled movement of the muscles). In some people nausea can happen, sometimes leading to vomiting. Diarrhea can also occur but is more rare. Changes in the mood have also been described including depression, irritability and loss of sexual appetite in women. A craving of salty foods is common, this is due to the loss of salts. Low blood sugar or hypoglycemia can also happen. In women, menstrual periods might become irregular or even stop.

The symptoms of acute adrenal insufficiency or addisonian crisis are more severe. They include severe pain in the lower back, abdomen or legs, pronounce vomiting and diarrhea potentially causing dehydratation, sudden loss of strenght, and low blood pressure that can cause loss of consciousness. An addisonian crisis is an emergency that needs immediate medical attention, the consequences can be fatal if left untreated. An acute adrenal insufficiency is usually triggered by an accident, trauma, surgery or severe infection.

Name Description
Dehydration Dehydration
Low blood pressure Low blood pressure
Loss of consciousness Loss of consciousness
Hyponatraemia low blood sodium levels
Skin discoloration Skin darkening around the joints, back and gums.

Diagnosis is sometimes difficult to obtain because most of the symptoms are not specific to Addison´s disease (can also occur in other medical conditions). A detailed review of the medical history of the patient and the presence of characteristic findings (like the darkening of the skin) can point to Addison´s disease. In most cases, the diagnosis is made by chance during a routine exam when a regular blood test shows increased levels of potassium or reduced levels of sodium.

Biochemical testing can help confirm an Addison´s disease diagnosis.

The ACTH stimulation test can confirm a primary adrenal insufficiency. In this test, a synthetic version of the hormone ACTH is injected in the blood and the levels of cortisol are measured before and after the injection. If the levels of cortisol do not increase after the ACTH administration, it indicates that the adrenal glands are damaged and unable to produce hormones.

If the adrenal glands are able to respond and produce hormones after ACTH administration, an insulin-induced hypoglycemia test can be done to measure the pituitary gland function. In this case, a fast-acting insulin is administered and the levels of both cortisol and glucose are monitored in blood before and after its injection. Insulin should cause a drop in the levels of glucose and increased levels of cortisol. If this does not occur, a pituitary disease can be the cause of secondary adrenal insufficiency.

Sometimes, imaging test can be performed to check the size of the adrenal glands and to look for structural abnormalities in them that can explain the cause of the adrenal insufficiency. The most common technique is a computerized tomography or CT scan, an especial X-ray equipment that creates detailed pictures of organs or areas inside the body.

The treatment of Addison´s disease requires the administration of the hormones that the adrenal glands are not able to produce, this is known as hormone replacement therapy. Usually oral corticosteroids (hydrocortisone, prednisone or cortisone) are prescribed to replace cortisol. If vomiting is an issue, corticosteroid injections might be needed. The drug fludrocortisone replaces the hormone aldosterone. The dosage of these drugs is different in between different individuals and should be determined for each person. The dosage may be increase during events known to trigger an acute crisis like infection, trauma, surgery or other stressful situations. This is a long-life treatment that can not be abandoned.

An acute adrenal crisis requires the immediate injection in the blood stream (intravenous) of high-dose hydrocortisone and salt water to replace the lost salts. Sometimes other drugs to increase the blood pressure called vasopressors are required.

People with Addison´s disease can have a normal life provided that they continue to take their hormone replacement medication. There are no specific restrictions and the lifespan is not affected.

Tips or Suggestions of Addison's Disease has not been added yet.

NADF. 2018. National Adrenal Disease Foundation. [ONLINE] Available at: http://www.nadf.us/adrenal-diseases/addisons-disease/. [Accessed 4 November 2018].

Eileen K. Corrigan. 2018. Pituitary Network Association. [ONLINE] Available at:https://pituitary.org/knowledge-base/disorders/adrenal-insuffieciency-addison-s-disease. [Accessed 4 November 2018].

Mayo Clinic. 2018. Mayo Clinic. [ONLINE] Available at: https://www.mayoclinic.org/diseases-conditions/addisons-disease/symptoms-causes/syc-20350293. [Accessed 4 November 2018].

NORD. 2018. National Organization for Rare Disorders. [ONLINE] Available at:https://rarediseases.org/rare-diseases/addisons-disease/. [Accessed 4 November 2018].

QR Code for Management of Adrenal Crisis Created by RareShareElena
Last updated 8 May 2018, 12:47 AM

secondary addisons Created by kelleeelmerick
Last updated 11 May 2012, 02:43 AM

Posted by kelleeelmerick
11 May 2012, 02:43 AM

Just got secondary or pituitary Addisons diagnosis. Anyone else have that form? Thank you.

Pigmentation Created by RH90
Last updated 6 Apr 2012, 10:36 PM

Posted by RH90
6 Apr 2012, 10:36 PM

Just thought I'd ask if people could share their own experiences. I've just turned 22 and for over a year I've been developing vitiligo, strangely it's been on my nipples. They have always been quite large and very dark and they are noticeably white in patches now, has anyone had this? Also, stupidly for a while now (years) I have been under-dosing on my medication and have developed a paler colour. Even though I've been told by my doctor to expect to go darker if I'm not getting enough Hydrocortisone/Fludrocortisone . My skin has always been noticeably yellow, anyone else got a yellow ting to their skin colour?

Greetings and salutations Created by dgimple1
Last updated 13 Dec 2010, 03:06 PM

Posted by dgimple1
13 Dec 2010, 03:06 PM

Thanks for the heads up. All the posts on this site seem pretty old, but it's a good idea. I updated some of the information on the basics of Addison's. Our biggest concern right now is finding a doctor that has experience with children with Addisons. we've met with three doctors here in town (Columbus, Ohio) and plan on heading down to Cincinnati to meet with the pediatric endocrinology department. Glad to hear it's manageable. We just need to get things locked down from a medication standpoint and hopefully move forward.

Posted by lfedeli0420
13 Dec 2010, 02:59 PM

Hi dgimple1, As far as I can tell, this site doesn't get a whole lot of traffic. Little traffic for those with Addison's any how. I'm sorry to hear your son was diagnosed and only 9 years old. I was diagnosed at age 13 with Addison's and I know how frustrating and scary it can be as a child when diagnosed with something no one really knows a whole lot about. Anyhow, I'm now a parent and can understand both the parent's side of dealing with a child diagnosed and the child's point of view. If you ever want to chat or ask questions, let me know. I've delt with Addison's for 22 yrs now and have had complications crop up through out the years. Good luck to you and your family. I hope doctor's are able to find a medication your son's body will absorb. Take Care, Leah Fedeli

Posted by dgimple1
9 Dec 2010, 08:23 PM

Found this site recently and wasn't sure how often it saw traffic. My nine year old son was diagnosed with Addison's disease about seven months ago and we've been trying to get our hands around it. Normally, a daily dose of cortisone will help to regulate everything but his body is not absorbing the medication. We've moved on to prednazone but it didn't work as well as we would think. Still running additional tests.

vitamin b12 deficiency Created by sunlover
Last updated 4 Oct 2010, 06:22 PM

Posted by lfedeli0420
4 Oct 2010, 06:22 PM

Hi, I too have hypothyroidism and addison's disease and a previous endo of mine put me on b12 shots to be taken monthly. They worked fine, but I discontinued the shots after about a year. No specific reason I stopped, but I will also say that I couldn't really tell a difference when taking the shots and when not taking the shots. Not sure if I have a vitamin D deficency, but I don't drink milk, so I probably do. How did you end up with Addison's? Just curious. Never met anyone with Addison's and I was diagnosed about 16 years ago, when in the 8th grade. Leah

Posted by sunlover
22 Jul 2009, 12:11 AM

I have primary Addison's disease. I was diagnosed in 1999. I also have hypothyroidism, I am doing well. I recently got a new endo who said I have vitamin b12 deficiency. I am giving myself shots for this once a month. Anyone have this same experience? How about vitamin D defiency? How common is this to happen?

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ALD Life is a patient support group and registered charity in the UK, we support patients and their families affected by Adrenoleukoystrophy (ALD) and Adrenomyelineuropathy (AMN) as well as patients with Addison's disease which in some cases will be caused by ALD.

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QR Code for Management of Adrenal Crisis

Created by RareShareElena | Last updated 8 May 2018, 12:47 AM

secondary addisons

Created by kelleeelmerick | Last updated 11 May 2012, 02:43 AM

Pigmentation

Created by RH90 | Last updated 6 Apr 2012, 10:36 PM

Greetings and salutations

Created by dgimple1 | Last updated 13 Dec 2010, 03:06 PM

vitamin b12 deficiency

Created by sunlover | Last updated 4 Oct 2010, 06:22 PM


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