Definition

Addison's disease, commonly known as primary adrenal insufficiency or hypoadrenalism, is an uncommon condition affecting the adrenal glands. The adrenal glands are a pair of small glands located superior to the kidneys. The glands are responsible for producing two crucial hormones, cortisol and aldosterone. Addison's disease impairs the functioning of the adrenal glands, leading to a malfunction in the synthesis of the chemicals cortisol and aldosterone.

The body releases cortisol as a response to stress. Cortisol regulates blood pressure, cardiac activity, the immune system, and blood glucose levels. Aldosterone modulates the balance of sodium and potassium in the circulation while regulating urine volume.

Causes

Addison's disease is characterized by impairment of the adrenal glands' outer layer (adrenal cortex), resulting in a decrease in the hormone levels produced by the adrenal glands.

Immune system disorders are the primary cause of Addison's disease. The immune system is the body's protective mechanism for fighting infection and disease. The immune system generates antibodies, a protein class that eliminates contaminants and pathogens. If there is a disruption in the immune system, the body's immune cells may begin attacking healthy tissues and organs.

This autoimmune disease leads to the body's immune cells launching an offensive against the adrenal glands, resulting in significant damage to the adrenal cortex. When 90% of the adrenal cortex is impaired, the adrenal glands cannot adequately synthesize cortisol and aldosterone chemicals, essential for fulfilling the body's needs. As adrenal hormone levels decrease, the symptoms of Addison's disease may develop.

The etiology of immune system problems in specific individuals remains unknown despite the observed familial predisposition to such conditions. Additional conditions that could potentially damage the adrenal glands include tuberculosis, adrenal gland infection, metastatic cancer affecting the adrenal glands, or hemorrhaging within the glands.

Risk Factor

Even though Addison's disease may happen to anyone of any age, the majority of cases are between the ages of 30 and 50. Women are more probable than men. Other medical disorders that increase the risk of autoimmune Addison's disease include:

• Type 1 diabetes mellitus
• Chronic thyroiditis an inflammatory condition of the thyroid gland
• Vitiligo (depigmentation of the skin), etc

Symptoms

Symptoms associated with Addison's disease typically occur gradually over several months. Initial minor symptoms commonly lead patients to ignore the problem. Therefore, additional symptoms of Addison's disease may be difficult to identify. Symptoms of a disease or injury may start to become more severe.

Several symptoms that are associated with Addison's disease are as follows:

• Fatigue or extreme lethargy
• Muscle weakness and cramping
• Dysphoria or irritability
• Loss of appetite and weight loss
• Polydipsia
• Dizziness, particularly at standing positions
• Hypotension
• Hyperpigmentation
• Darkening lips and gums
• Desire for salty foods
• Abdominal pain and headache
• Polyuria
• Inability to concentrate
• Hypoglycemia

Diagnosis

The doctor will interview you regarding the symptoms and medical history to diagnose Addison's disease, including a familial medical history, especially if the family member has an autoimmune disease.

The doctor will carry out a comprehensive physical examination. Blood pressure, pulse, breathing, and temperature are vital signs that will be evaluated during this procedure. Hypotension might be observed, particularly during postural changes from a supine to an upright position. Hyperpigmentation can also manifest in dark skin patches, particularly in regions such as the palmar creases and elbow creases. The lips and gums indicate a darker appearance as well. Nevertheless, these hyperpigmented patches are not universal in all cases of Addison's disease.

Additional tests support the diagnosis. Doctors may recommend these tests:

• Evaluate the levels of electrolytes, specifically sodium and potassium, within the body
• An analysis of the hormones cortisol, aldosterone, and ACTH
• Monitoring blood glucose levels
• Adrenal antibody analysis

If you experience low blood cortisol or intense symptoms of Addison's disease, a synacthen stimulation test is needed. Synachten is a synthetic ACTH hormone. The brain's pituitary gland produces ACTH, which causes the adrenal glands to release cortisol and aldosterone. Synacthen should behave like ACTH on the adrenal glands. High ACTH but low cortisol and aldosterone levels usually indicate Addison's disease.

Management

Addison's disease is treated with hormone replacement. Lifelong medication is required.

Treatments may effectively manage the symptoms of Addison's disease. Most individuals with this condition may live active lives with minimal limitations. Occasionally, the underlying cause of Addison's disease might be treated. Anti-tuberculosis treatment for a minimum of six months is necessary to treat Addison's disease resulting from TB infection effectively.

Nevertheless, the majority of Addison's disease cases are associated with incurable immune system disorders.

Complications

People with Addison's disease must be cautious of the possibility of a sudden exacerbation of symptoms. This disease, known as an adrenal crisis, develops once the cortisol levels decrease significantly. Significant physical stress following a surgical procedure or another condition, such as an infection, may lead to an adrenal crisis.

The rapid onset of severe symptoms characterizes adrenal crisis in individuals with Addison's disease. Signs of an adrenal crisis include the following:

• Extreme dehydration
• Dizziness
• Hyperhidrosis
• Rapid, shallow breathing
• Pallid, frigid, and moist skin
• Hypotension
• Severe vomiting and diarrhea
• Abdominal pain and headache
• Fatigue and severe muscle weakness
• Unconsciousness

Adrenal crisis is an emergency condition that requires immediate treatment. Inadequate and late medical intervention may result in a condition of coma and mortality. Furthermore, if therapy is delayed, brain tissue may not receive sufficient oxygen, leading to permanent disability.

Prevention

Currently, there are no confirmed prophylactic treatments to avoid the onset of Addison's disease. Adrenal crisis is preventable, however, in patients with Addison's disease by recognizing the symptoms and seeking medical attention immediately if they develop severe complaints.

When to see a doctor?

Consult a general practitioner or specialist in internal medicine if the symptoms above persist despite treatment for an extended period. Further investigation of these symptoms is necessary.

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