Addison’s disease: Symptoms, Causes, Treatment

The Addison’s disease (AD) or primary adrenal insufficiency is an autoimmune disease characterized by dysfunction or hypo function of the adrenal cortex (Roy Gomez et al., 2013).

This disorder therefore occurs when the body begins to produce an insufficient amount of several hormones that produce the adrenal glands. Specifically, these glands produce high amounts of cortical and reduced aldosterone (Mayo Clinic, 2015).

Addison’s disease

It is a rare disease in childhood, but it can be potentially fatal if it is not diagnosed early (Roy Gomez et al., 2013).

Addison's Disease

In general, symptoms appear progressively with asthenia, anorexia, weight loss, vomiting, hypoglycemia, among others (Roy Gomez et al., 2013) and are usually the result of infectious diseases (Cassava et al., 2006).

What are the functions of the adrenal glands?

The adrenal glands are located in the back of the two kidneys (NHI, 2014).

The outer region, the cortex, is responsible for the production of different steroid hormones such as cortical, aldosterone and different hormones that can be transformed into testosterone (NHI, 2014).

On the other hand, the inner region, the medulla, is responsible for the production of epinephrine and nor epinephrine (NHI, 2014).

The efficient production of this type of hormone is essential for the optimal functioning of our body. When these glands or other structures alter the production of one or several essential hormones for the body, various pathologies may develop (NHI, 2014).

Primary disorders such as infectious diseases, autoimmune diseases, neoplasm’s or autoimmune diseases can significantly alter the hormonal production of the adrenal glands (NHI, 2014).

The adrenal hormones play an essential role in the functioning of the body, regulating blood pressure, metabolism, and use of nutrients or different body’s responses to stress (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

In relation to the main hormones that are altered in Addison’s disease:

Cortical

This hormone belongs to the group of glucocorticoids that affects almost all body tissues and organs (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

The cortical is a substance that promotes the body’s response to stress. On the other hand, it also helps maintain blood pressure, heart function, blood glucose level (Cleveland Clinic, 2015).

On the other hand, cortical also helps regulate the immune system, when the body recognizes a foreign agent and defends itself against bacteria, viruses and other harmful substances (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

The production of optimal and balanced amounts of cortical is regulated by the hypothalamus and pituitary gland (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

The hypothalamus releases a hormone called corticotrophin-releasing hormone (HLC), which signals the pituitary gland to release adrenocorticotropic hormone (ACTH) that stimulates the adrenal glands to produce cortical (National Institute of Diabetes and Digestive and Kidney Diseases, 2014). ).

Aldosterone

The adoslterona is a kind of hormone which belongs to the mineral corticoid and is also produced by the adrenal glands (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

Aldosterone is related to balance of sodium and blood potassium. In addition, it controls the amount of fluids the kidneys must remove (urine) (Cleveland Clinic, 2015).

The decrease in sodium in the blood can cause both a decrease in blood pressure and total blood volume. In addition, it may also lead to hoponatremia (decreased sodium causing symptoms of confusion, tiredness, fatigue, muscle spasms and / or convulsions) (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

Dehydroepiandrosterone

Although it is a substance that is less affected by the development of Addison’s disease, dehydroepiandrosterone (DHEA) is another hormone produced by the adrenal glands (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

Our body uses dehydroepiandrosterone (DHEA) for the production of sex hormones, androgens and estrogens. When adrenal insufficiency occurs, sufficient amounts of dehydroepiandrosterone (DHEA) (National Institute of Diabetes and Digestive and Kidney Diseases, 2014) may not be produced.

Although healthy men and women derive most androgen and estrogen from sexual structures, specifically in women and adolescents, dehydroepiandrosterone (DHEA) deficiencies can lead to loss of pubic hair, depression or loss of sexual interest (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

Characteristics of Addison’s disease

Adrenal insufficiency is an endocrine / hormonal disorder that occurs when the adrenal glands do not produce enough of certain hormones (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

Adrenal insufficiency can be primary or secondary. However, the term Addison’s disease is commonly used to denote primary adrenal insufficiency (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

Addison disease or primary adrenal insufficiency is a pathology that is related to hypo function of the adrenal glands (Cleveland Clinic, 2015). This alteration can lead to a low production of cortical and aldosterone or even the inactivation of the adrenal glands (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

However, we can also distinguish a type of secondary adrenal insufficiency that affects the functioning of the pituitary gland (located at brain level). There is a decrease in the secretion of adrenocorticotropic hormone, which is responsible for activating the adrenal glands for the production of the hormones mentioned above (Cleveland Clinic, 2015).

About us

Secondary adrenal insufficiency is much more frequent than Addison’s disease or primary adrenal insufficiency (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

Approximately 110-144 individuals out of every one million people suffer from Addison’s disease (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).


The incidence of Addison’s disease is estimated at 0.8-1.4 cases per 100,000 population / year. In addition, it is a rare disease in the pediatric age (Roy Gomez et al., 2013)

In the United States, Addison’s disease affects 1 in 100,000 people and occurs in both men and women in a similar way.

Although it may occur in all age groups, it is more common to appear between 30 and 50 years of age (Cleveland Clinic, 2015).

Symptoms and signs

Usually the symptoms of Addison’s disease develop gradually over several months (Mayo Clinic, 2015).

The most frequent symptoms of adrenal insufficiency are (National Institute of Diabetes and Digestive and Kidney Diseases, 2014):

  • Chronic and long-term fatigue.
  • Muscular weakness.
  • Abdominal pain.

Other symptoms that are also commonly seen in individuals with Addison’s disease are (Mayo Clinic, 2015):

  • Loss of weight and significant decrease in appetite.
  • Hyper pigmentation or darkening of the skin.
  • Reduced blood pressure, fainting.
  • Hypoglycemia or low blood glucose levels.
  • Need or desire to ingest salt.
  • Nausea, diarrhea, vomiting.
  • Abdominal pain and discomfort
  • Muscle and joint pain and discomfort.
  • Irritability.
  • Depressive symptomatology.
  • Loss of hair (when there is a dysfunction of sex hormones in women.

In general, slowly progressing symptoms have to be ignored until an event with a high level of stress, such as surgery, illness, serious injury or pregnancy worsens its clinical course (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

In other cases, symptoms of this pathology may occur suddenly, leading to an acute Addison’s disease crisis or Addison’s crisis (Mayo Clinic, 2015):

  • Acute renal failure.
  • Pain in the abdomen, lower back and legs.
  • Vomiting and severe diarrhea.
  • Dehydration.
  • Significant reduction in blood pressure.
  • High levels of potassium (hyperkalemia) and low sodium content (hyponatremia).

Causes

Autoimmune disorders and disorders are the main cause of most cases of Addison’s disease.

However, some infections and / or drugs may also contribute to the development of this pathology (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

Autoimmune Disorders

Addison’s disease may occur as a consequence of an autoimmune response of the body (Cleveland Clinic, 2015).

Approximately 80% of cases of Addison’s disease are caused by an immunological type disorder (National Institute of Diabetes and Digestive and Kidney Diseases, 2014), which occurs when the immune system “attacks” to their own organs and tissues (Cleveland Clinic, 2015).

In Addison’s disease, the immune system attacks the outside of the adrenal glands where cortical and aldosterone are produced (Cleveland Clinic, 2015).

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The autoimmune cause of Addison’s disease occurs primarily in middle-aged women (National Institute of Diabetes and Digestive and Kidney Diseases, 2014)

Infections

Some infectious causes that may lead to the development of Addison’s disease have also been described (Cleveland Clinic, 2015).

Tuberculosis is one of these infections that can damage or destroy the adrenal glands. Approximately 10-15% of cases of Addison’s disease have their origin in tuberculosis (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

On the other hand, recent clinical investigations have indicated an increase of the cases of Addison’s disease as a result of the cytomegalovirus (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

Cytomegalovirus does not usually cause symptoms in healthy people, however, if it affects babies during gestation or people who have a weakened immune system (VHI) (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

One of the causes of meningitis (Neustria meningitides) or fungal infections can also lead to the development of Addison’s disease (National Institute of Diabetes and Digestive and Kidney Diseases, 2014).

Other (less common) causes that may lead to the development of Addison’s disease have also been described (Cleveland Clinic, 2015):

  • Injuries to the adrenal glands.
  • Oncologic metastasis.
  • Bleeding from the adrenal glands.
  • Surgical removal of the adrenal glands.
  • Amyloidosis (abnormal accumulation of certain proteins)
  • Genetic defects.

Treatments

All therapeutic interventions for Addison’s disease focus on the use of hormone replacement therapies to compensate and correct hormone levels (Mayo Clinic, 2015):

Some of the treatment options are (Mayo Clinic, 2015):

  • Oral corticosteroids or injections: hydrocortisone, prednisone or cortisone acetate is often used to replace cortical. In the case of aldosterone deficiency, some specialists recommend the use of fludrocortisones.

On the other hand, abundant amounts of sodium are also recommended, especially during intense exercise, warm weather, or intense hormonal diarrhea and vomiting (Mayo Clinic, 2015).

In addition, it should be noted that when an Addison crisis occurs the person’s survival is severely threatened: low blood pressure, blood glucose or high levels of potassium in the blood (Mayo Clinic, 2015).

When an Addison crisis develops, getting medical care is essential. Treatment in the acute phase generally includes (Mayo Clinic, 2015):

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