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Autoimmune Addison’s Disease - An Overview

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Autoimmune Addison’s disease is a rare autoimmune-mediated disorder characterized by impaired functioning of the adrenal glands. Read the article to know more.

Medically reviewed by

Dr. Shaikh Sadaf

Published At July 18, 2023
Reviewed AtJuly 25, 2023

Introduction

Addison’s disease is an uncommon chronic disorder that results from the impaired functioning of an adrenal cortex, thereby leading to a deficient production of hormones such as glucocorticoids, mineralocorticoids, and male hormones. It has been reported by the researchers that autoimmune Addison’s disease is the most common cause of the disease in adults, accounting for more than 85 percent of cases, followed by tuberculosis infection in approximately 11 percent of patients, and the remaining four percent of patients are affected either due to vascular, malignant or hereditary forms.

What Is Autoimmune Addison's Disease?

Autoimmune Addison’s disease is an uncommon autoimmune disease that alters the function of the adrenal glands. The adrenal glands are tiny hormone-synthesizing glands that are located on top of each renal capsule. It is subclassified as an autoimmune disease because it is the result of a malfunctioning immune system that invades these adrenal glands. The production of several hormones is therefore interrupted, thereby affecting several body organs.

What Are the Other Synonyms of Autoimmune Addison's Disease?

  • Autoimmune Addison disease.

  • Autoimmune adrenalitis.

  • Classic Addison’s disease.

  • Primary Addison’s disease.

What Are the Signs and Symptoms of Autoimmune Addison's Disease?

The clinical signs and symptoms of autoimmune Addison’s disease can appear at any age, but mostly they begin to show between the third and fifth decades of life. Some of the common symptoms of the disorder include:

  • Tiredness (extreme fatigue).

  • Nausea and vomiting.

  • Loss of appetite.

  • Loss of weight.

  • Low blood pressure.

  • Dizziness on standing up suddenly.

  • Muscle pain.

  • Craving for salty food products.

  • Hyperpigmentation is a characteristic feature of autoimmune Addison’s disease that is characterized by abnormally dark, blackish areas of skin, especially in areas such as the armpits, elbows, knuckles, and creases of the palm. These areas experience a lot of friction. Even the labial mucosa and the lips can turn unusually dark.

  • Women might lose their pubic hair and underarm hair due to a hormonal imbalance created. These hormones are responsible for developing secondary sexual characteristics in humans.

  • Hypoglycemia (low levels of sugar) is present in the blood.

  • Low levels of sodium (hyponatremia) are observed in the blood.

  • High levels of potassium (hyperkalemia) are observed in the blood.

  • Anemia may sometimes be seen in the affected individuals.

  • An increase in the number of white blood cells, particularly eosinophils, is also seen in the blood.

  • Autoimmune Addison’s disease can cause a life-threatening crisis of the adrenal glands. The condition can lead to severe nausea and vomiting, pain in the abdomen, muscle cramps in the back or legs, and extremely low blood pressure leading to shock. Such a crisis can usually be triggered in cases of surgery, trauma, or bacterial or viral infections.

What Is the Frequency of Autoimmune Addison's Disease?

Addison's disease is found in approximately 10 to 15 people out of 100,000 people having European descent. The autoimmune form of Addison’s disease is the most frequently present form in developed countries, that account for more than 90 percent of the disease cases.

What Are the Causes of Autoimmune Addison's Disease?

The etiology of autoimmune Addison’s disease is not completely known. A multifactorial etiology of environmental and hereditary factors plays a major role in disease pathogenesis, and multiple gene mutations are found to be responsible for developing the serious condition.

The genes also participate in the immune reactions of the body. They have been found to be related to autoimmune Addison’s disease also. Human leukocyte antigen complex genes have been found to be most commonly associated with the disease. The human leukocyte antigen complex helps to differentiate between the proteins that are synthesized by the body from the proteins produced by foreign agents like viruses and bacteria. Various phenotypic variations are present in each human leukocyte antigen gene. This genetic arrangement allows the immune system of every individual to react differently to the different foreign proteins that are present. The HLA-DRB1 gene is the most common predisposing factor responsible for causing autoimmune Addison’s disease.

In autoimmune Addison’s disease, a typical adrenal gland protein called 21-hydroxylase is responsible for initiating an immune response. This adrenal gland protein is mainly responsible for the production of certain hormones in the adrenal glands. The immune attack is prolonged and usually initiated by 21-hydroxylase, which leads to damage to the adrenal glands, in particular the adrenal cortex, thereby preventing the production of hormones. It is this adrenal insufficiency (a deficiency of adrenal hormones) that leads to the disruption of various functions in the body, thereby causing low blood sugar, low sodium levels in the blood, hypotension, muscular cramps, skin darkening, and other clinical features of autoimmune Addison’s disease.

How to Diagnose Autoimmune Addison's Disease?

The healthcare professional will ask about the medical history and the symptoms experienced by the patient. He will carry out a physical examination, and he might suggest some laboratory tests to check the serum potassium and serum sodium levels. The healthcare professional may also suggest certain imaging tests and measure the hormonal levels.

How Is Autoimmune Addison's Disease Treated?

The doctor prescribes medications that will help to regulate your adrenal glands.

It is very important to follow the treatment plan suggested by your doctor. If left untreated, Addison’s disease can progress to a life-threatening condition called Addisonian crisis. In this condition, the doctor may suggest medications to treat the Addisonian crisis first.

Medications - A combination of glucocorticoid medications (anti-inflammatory drugs) to improve the health of an individual is generally prescribed. These medications need to be taken for the rest of the life, and not a single dose can be missed. Hormone replacement therapy may also be prescribed to replace hormones that are not being produced by the adrenal glands.

Conclusion

Autoimmune Addison’s disease is a serious disorder affecting the functions of the adrenal glands. Individuals should seek the advice of a doctor if they are experiencing the symptoms of the disease. Follow-up on a regular basis is required to improve overall health. Always keep an emergency kit of medicines at home handy at all times. Do not take the condition lightly.

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Dr. Shaikh Sadaf
Dr. Shaikh Sadaf

Endocrinology

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addison's diseaseautoimmune disorder
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