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Addison’s Disease (Adrenal Cortex Insufficiency)

This disease caused by the destruction of the adrenal cortex was first described in 1855 by Dr Thomas Addison, a British physician.The adjective term “Addisonian” explains features of this condition, and patients suffering Addison’s disease. Addison’s disease is a rare endocrine disorder that occurs when the when the adrenal glands do not produce enough amounts of certain hormones especially glucocorticoids and often mineralocorticoids. In Addison’s disease, the adrenal glands produce very little cortisol (corticosteroid hormone or glucocorticoid ) and also the amount of aldosterone produced is insufficient. Addison’s disease which is also referred to as chronic adrenal insufficiency, hypocortisolism, or hypocorticism occurs in people of all ages. This disease affects both males and females and if it is not treated early it can prove to be deadly.

Clinical Signs and Symptoms

Generally the symptoms of Addison’s disease develops gradually over several months. The main symptoms include

  • General weakness
  • Anemia
  • Low blood pressure
  • Loss of weight
  • Hypoglycemia (Low Blood Sugar)
  • Extreme sensitivity to insulin
  • Excessive loss of sodium and chloride in the urine
  • Impaired elimination of water by the kidneys
  • Hyperpigmentation - A characteristic pigmentation due to accomplished of the brown pigment melanin is observed especially in exposed areas (hands and face) and sites exposed to friction. In simple words it is the darkening of skin.
  • Loss of appetite
  • Nausea
  • Loose motion
  • Depression

Metabolic Features

The important metabolic features are

  • Hypoglycemia and increased sensitivity to insulin
  • Excretion of Na in urine and retention of K ion in tissues
  • Decreased excretion of water and
  • Reduced diuretic response after ingestion of water.

Causes

Addison’s disease occurs when the adrenal glands gets damaged and thereby produces less amounts of the hormone cortisol and often aldosterone as well. Adrenal glands have two sections namely medulla and cortex. The adrenaline-like hormones are produced by the interior medulla and the outer layer called cortex produces a group of hormones called corticosteroids. Corticosteroids are a class of steroid hormones which include glucocorticoids, mineralocorticoids and androgens. Androgens, also called androgenic hormones are the male sex hormones.

Primary adrenal insufficiency

In most cases Primary adrenal insufficiency is caused by the gradual destruction of the outer layer of the adrenal glands (adrenal cortex), by the body’s own immune system. This condition involving damage to the adrenal glands is known as primary adrenal insufficiency.

The adrenal glands fail to produce adrenocortical hormones as a result of autoimmune disease (body attacking itself). Addison’s disease are mainly caused by autoimmune disorders, in which the immune system produces antibodies which attack the body’s own organs and gradually destroy them. The immune system views the adrenal cortex as foreign body and tries to destroy it but the reason for this is unknown. As a result, the hormones glucocorticoid (cortisol) and mineralocorticoid (aldosterone) are lacking.

Other causes of adrenal gland failure include:

  • Tuberculosis
  • Polyendocrine deficiency syndrome
  • Chronic infections – mainly fungal infections
  • Amyloidosis
  • Adrenal cancer
  • Adrenal gland physical damage
  • Adrenal gland removal

Secondary adrenal insufficiency

Secondary adrenal insufficiency is more common than primary adrenal insufficiency and the reason is the lack of a hormone made by the pituitary gland called adrenocorticotropic hormone (ACTH). Adrenal insufficiency also occurs if the pituitary gland is diseased. ACTH stimulates the adrenal cortex to produce its hormones. Insufficient production of ACTH can lead to inadequate production of hormones by the adrenal glands even if the adrenal glands are not damaged.

Pituitary glands might be the underlying cause of Addison’s Disease:

  • Pituitary tumor
  • Pituitary tumor radiation treatment
  • Pituitary infection
  • Hypothalamus surgery or removal

Secondary adrenal insufficiency occurs when persons who take corticosteroids for treating chronic conditions, such as asthma suddenly stop taking corticosteroids.

Treatment for Addison’s Disease

Medical treatment of Addison’s disease involves replacing the hormones that are not produces by the adrenal glands. Cortisol is replaced orally by taking hydrocortisone once or twice everyday based on the patient’s condition. If the person has insufficient oral doses of a mineralocorticoid called fludrocortisone acetate (Florinef) should be taken once a day. Patients who undergo aldosterone replacement therapy should increase their daily salt intake. Generally patients with secondary adrenal insufficiency have enough aldosterone production and so they don’t require aldosterone replacement therapy.

Addisonian crisis

If Addison’s disease is left untreated then an addisonian crisis may be provoked when any type of physical stress such as injury or illness occurs.

When addisonian crisis occurs, low blood glucose, low blood pressure, and high potassium content may become dangerous to life. Standard therapy involves intravenous injections of hydrocortisone, saline (salt water), and dextrose (sugar). This treatment improves the patient’s condition very well. If the patient is aldosterone deficient he undergoes maintenance therapy taking oral doses of fludrocortisone acetate.

Surgery: If the patients suffers from chronic adrenal insufficiency surgery is done by giving general anesthesia and also treated with injections of hydrocortisone and saline.

If pregnant: If women gets pregnant with primary adrenal insufficiency then standard replacement therapy is done. During early stages of pregnancy she might often have nausea or vomiting which affects oral medication and so hormones injections are required. During the time of delivery the treatment done is similar to that of patients who need surgery. After delivery, the dose is gradually reduced and ten days after child birth the maintenance doses of hydrocortisone and fludrocortisone are increased to usual level.

Home treatment through proper Nutrition

The treatment consists in the administration of cortisone which corrects all the metabolic disorders.

  • Calories: The calorie intake should be about 25% excess of the daily requirements as the patients are usually under weight.
  • Proteins: The protein intake should be about 50% excess of the daily requirements
  • Minerals: Since excess of sodium is excreted in urine, the sodium chloride intake should be increased to make up the loss. Since potassium ion is retained, the intake of potassium should be restricted to the minimum.
  • Vitamins: The intake of all vitamins should be adequate to meet the daily requirements. This can be achieved by the ingestion of a multi-vitamin diet.

The home treatment is just an additional support to the main treatment. Even if you have recovered from Addison’s disease you can follow this home treatment to increase the overall nutrition level in your body.

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