Addison’s disease: what it is, causes, symptoms and treatment

What is Addison’s disease?

Common in age 40, but developing at any age, Addison’s disease, also known as primary adrenal insufficiency or chronic primary adrenocortical insufficiency , affects both men and women.

About 4 out of every 100 thousand people are affected, making it noticeable in situations of trauma or stress . In pediatric age, the incidences are even rarer.

In 70% of cases the cause is not known, however, it is assumed that there is a change in the adrenal (or adrenal) glands caused by autoimmune diseases , that is, the immune system itself attacks and extinguishes the adrenal cortex.

The other 30% are from pathologies, cancer or infections. In babies or children, the problem in the adrenal glands is due to genetic abnormalities. The most common symptoms are weakness, dizziness when standing up and dark spots on the body.

Addison’s disease takes its name from the English physician Thomas Addison, who first identified it in 1855. It can be found in the International Classification of Diseases (ICD-10) under the code E27.1.

Adrenal insufficiency: primary or secondary?

Adrenal insufficiency can be categorized as primary or secondary. However, its causes are distinct:

Primary adrenal insufficiency

The primary adrenal disorder occurs due to the destruction or inactivation of glandular functions through autoimmune diseases, cancers or infections, which characterizes Addison’s disease.

Secondary adrenal insufficiency

Secondary adrenal insufficiency is the name given to a problem similar to Addison’s disease. It is not Addison’s disease itself, because the cause of the problem does not originate in the adrenal glands, but in the brain.

In this case, there is a problem with the pituitary (or pituitary) gland. It is the one who produces the adrenocorticotrophic hormone (ACTH), responsible for boosting the production of other hormones by the adrenal cortex.

Therefore, despite the fact that the adrenal glands are not damaged, hormonal production can be compromised due to changes in hormone production resulting from the abnormal performance of the pituitary gland.

As a result, the concentration of water, sodium and potassium in the body undergoes changes, in addition to affecting the body’s dominance under blood pressure and the reaction in stressful situations.

The absence of androgens – a steroid hormone that drives or monitors the development and maintenance of male characteristics and precursors of estrogens (female hormones) – such as dehydroepiandrosterone (DHEA), can cause the loss of body hair in women. In men, testosterone supplies this loss.

Among the causes pointed to the appearance of the disorder, are:

• Corticosteroids for treatment of chronic diseases, such as asthma and arthritis, and which stop taking;
• Tuberculosis;
• Infection by fungi or viruses;
• Primary tumor (originated from the organ itself) or secondary tumor (which started from another organ);
• Hemorrhage;
• Trombose adrenal;
• Amyloidosis;
• Sarcoidosis (the appearance of inflammatory cells in various regions of the body);
• Hemochromatosis (accumulation of iron in the body);

Such diseases are more frequent in older people, and can affect both men and women.

Pathophysiology

The adrenal glands are located over the kidneys and are made up of different tissues. One of them constitutes the external part of the gland, the cortex. The other, on the other hand, forms its interior, the medulla.

Adrenal medulla

The adrenal medulla is responsible for the production of two hormones: adrenaline and norepinephrine . The first one allows an immediate reaction of the organism in dangerous situations; the second aims to maintain blood pressure at normal levels.

Adrenal cortex

The adrenal cortex produces steroid hormones , also known as corticosteroids.

The most important are glucocorticoids and mineralocorticoids. Problems like Addison’s disease can cause a deficiency in the production of these two hormones.

Glucocorticoids

Glucocorticoids act in the metabolism of carbohydrates , proteins and fats. They work by breaking down these molecules into glucose. They also have anti-inflammatory and immunosuppressive action (they reduce the activity or efficiency of the immune system).

Glucose serves as energy in stressful conditions. The lack of this hormone in the body can cause hypotension (lower than normal pressure), insulin sensitivity and problems related to carbohydrates, fats and proteins.

Among the main varieties of glucocorticoids is cortisol, also called hydrocortisone. The component allows certain substances from the blood to enter tissues that are next to it.

Its absence brings complications such as hypoglycemia (low blood sugar) and reduction of hepatic glycogen, which constantly maintain the amount of blood glucose.

Mineralocorticoids

Mineralocorticoids control the levels of water and salts in the body. Changes in these components result in low sodium condensation (insufficient aldosterone, which makes sodium reabsorption) and high potassium.

As a consequence, there may be severe dehydration, plasma hypertonicity (when the sodium concentration is greater than 160 mEq / L), acidosis (blood pH below 7.35), hypotension and circulatory instability.

Causes

Alterations in the structure or even the destruction of the outer layer of the adrenal glands decrease the concentration of hormones produced by it, causing Addison’s disease. The main causes are:

Immune system

The immune system is made up of cells, tissues and organs that work together to protect the human body from infections and diseases.

When foreign substances enter the body (antigens), they cause the production of antibodies. This is a natural defense response of the organism to attack the cause of the problem.

However, if there is a disturbance with the immune system, it will attack its own healthy tissue and organs. This condition is known as autoimmune disease.

In this case, 90% of the adrenal cortex is destroyed, making the production of steroid hormones, cortisol and aldosterone scarce.

Cancer

Cancer cells spread quickly and destroy tissue in the body, reaching the adrenal glands.

Amyloidosis

Amyloidosis is a rare problem in which the accumulation of amyloid (protein produced by bone marrow cells) occurs in various tissues and organs, including the adrenal glands, altering its functioning.

Adrenoleucodistrofia (ALD)

An unusual disease, Adrenoleukodystrophy (ALD), also called Lorenzo’s disease, damages the central nervous system and the adrenal glands.

Associated with the X chromosome (female), it is hereditary, passed from mother to child. 50% of women who have the problem are at risk of transmitting it to the child, especially boys.

Surgery

Surgical removal of the adrenal glands due to nodules or tumors can lead to complications, such as insufficient cortisol production.

Risk factors

There are other risk factors, such as some autoimmune diseases, that make Addison’s disease possible. Are they:

Anticoagulant medications

People who use anticoagulant drugs are more likely to develop the problem.

Genetics

According to studies, it may be related to cases in the family or when the person has other autoimmune diseases:

• Vitiligo: pathology that causes depigmentation in the skin due to the reduction or lack of melanocytes;
• Type 1 diabetes: production of little or no insulin. As a result, glucose stays in the blood instead of being used as energy;
• Thyroid hypoactivity (hypothyroidism): occurs when there is little thyroid hormone.

Infections

Some infections can lead to damage to the adrenal glands:

• Tuberculosis: infectious and transmissible disease through inhalation of aerosols (particulate particles suspended in the air) when sneezing, coughing or talking. The lung is the most affected, but other organs and systems in the body can be compromised, such as the adrenal glands;
• HIV: the virus affects the immune system, which defends the human body from diseases, which, in turn, damages the adrenal gland;
• Hemorrhagic fungal infections: related to meningitis (inflammation of the membranes that cover the brain and spinal cord) and sepsis (when the whole body reacts to an infection, leading to the organ’s inability), damage the adrenal glands.

Graves’ disease

Graves’ disease is caused by a disorder of the immune system, culminating in an excess of hormones produced by the thyroid.

It is primarily responsible for hyperthyroidism , especially in women aged 20 to 50, but it can occur at any age.

Testicular dysfunction

Testicular dysfunction occurs when there is excessive or insufficient production of androgens, testosterone and dihydrotestosterone. The emergence of the problem is related to drugs, diseases or trauma.

Pernicious anemia

The Anemia Pernicious, also known as microcytic anemia, pernicious anemia , congenital, juvenile pernicious anemia , or vitamin B12 deficiency anemia is not caused by the absorption of vitamin B12 in the small intestine lowering the levels of red blood cells.

Dermatite herpetiforme

The dermatitis herpetiformis, Duhring celiac disease or dermatitis herpetiformis, is perceived through rashes (blisters and lumps) itchy. People who have gluten intolerance are more likely to have dermatitis, however, it can be triggered in anyone.

Hypoparathyroidism

Hypothyroidism occurs due to the low production of parathyroid hormone (PTH), which balances the concentration of calcium and phosphorus in bones and blood, which can result in problems in bones, muscles, skin and nerve endings.

Treatments against Cushing’s syndrome

Cushing’s syndrome, also called hypercortisolism, is a condition characterized by the high level of cortisol in the body. Some of your treatments can cause Addison’s disease.

Symptoms

As it is similar to other pathologies, the symptoms of Addison’s disease may not be noticed immediately. They tend to progress gradually over months or years.

However, together with the stress caused by other illnesses or traumas, it can cause the condition to worsen abruptly.

Among the most common symptoms are dark spots and freckles on the skin, similar to tanning. They become evident when exposed to the sun, but they can appear in places that are not as noticeable, such as the gums.

Black people can also suffer from excess pigmentation, although the change is more difficult to see. The most affected parts of the body are: forehead, knees, elbows, scars, skin folds and creases (as in the palms of the hands).

Bluish black spots may appear around the nipples, lips, mouth, rectum, scrotum or vagina.

The other symptoms include:

• Fatigue;
• Muscle weakness and pain;
• Sleep out of the ordinary;
• Tiredness;
• Bad mood (mild depression);
• Irritability;
• Cold intolerance;
• Lack of appetite;
• Reduced libido;
• Weight loss;
• Urinating frequently;
• Being very thirsty;
• Dehydration;
• Do not digest food;
• Enjoo;
• Vomiting;
• Diarrhea.

Some women experience irregular menstrual cycles or the complete disappearance of menstruation. Conditions such as hypoglycemia (low blood sugar), nervousness and preference for salty foods appear mostly in children. In addition, teenagers may have their puberty delayed.

In aggravated states, the person with Addison’s disease may experience severe abdominal pain, low blood pressure (hypotension) – especially when standing, which can lead to dizziness and fainting -, have kidney failure and their condition progressing to a crisis adrenal.

Crise adrenal

The signs of Addison’s disease tend to develop gradually over months or years. However, together with stress, resulting from other illnesses or traumas, it can cause the condition to worsen abruptly.

This worsening is called an adrenal or Addisonian crisis.

The crisis can occur in people who do not manifest the symptoms or in an early stage of the disease. In this state, the effects include:

• Severe dehydration;
• Pale, cold and sweaty skin;
• Rapid and shallow breathing;
• Intense vomiting;
• Diarrhea;
• Headache;
• Marked muscle weakness;
• Severe drowsiness;
• Loss of consciousness.

Some conditions favor the incidence of adrenal crises:

• People with Addison’s disease;
• Surgeries in the adrenal glands;
• Injury to the pituitary gland (pituitary gland);
• Individuals who have adrenal insufficiency, but who do not take medication;
• Physical trauma or severe stress.

How is the diagnosis made?

Due to the insignificant signs of Addison’s disease, the doctor may not notice it immediately. A high level of stress can make symptoms more evident and anticipate a crisis.

The specialist in this area is the endocrinologist . The tests applied by him can be:

Bloodtests

Through a blood test, the specialist will be able to check the amount of cortisol or adrenocorticotrophic hormone (ACTH). People with Addison’s disease have ACTH greater than 22 pmol / L. There is no specific estimate for cortisol, but it is usually less than 83 nmol / L.

In addition, other components may be considered:

ACTH stimulation

The assessment of cortisol levels occurs through ACTH stimulation, before and after the procedure. In healthy patients, the concentration of cortisol increases (greater than 500 nmol / L) after contact with the adrenocorticotropic hormone, which does not occur in people with the disease.

Serum potassium test

In this analysis, the amount of potassium in the body is perceived. The disease is suspected due to high levels.

Serum sodium test

The purpose of the test is to measure sodium levels. People with the disorder tend to have low levels.

Glucose test

The test should be done on an empty stomach to determine the amount of glucose in the blood. If the amount is low, it is a sign of the disease.

Imaging exams

Imaging exams are performed when there is evidence of calcifications in the adrenals (accumulation of calcium, leaving the glands with a hardened aspect), renal or pulmonary tuberculosis .

Is Addison’s disease curable?

Addison’s disease has no cure . However, according to the assessment and the proper treatment, it is possible to have a normal life. The disorder brings complications and even death when the individual refuses medical intervention.

What is the treatment?

The treatment of Addison’s disease, when done correctly, is effective, allowing the patient to develop their daily activities normally. Related diseases can also be treated.

Tuberculosis, for example, can be treated with antibiotics for at least 6 months. However, disorders of the immune system cannot be cured.

Treatment consists of oral or intravenous drugs:

Medication

Due to the low production of hormones by the adrenal glands, replacement is recommended. One of the alternatives is the use of corticosteroids (steroids) to replace cortisol and aldosterone. They are usually presented in tablets, taken 2 to 3 times a day.

People in more aggravated states can receive cortisol intravenously or intramuscularly and then hydrocortisone tablets.

It may also be necessary to restore calcium and potassium in the body. Although it is not necessary, the replacement of dehydroepiandrosterone (DHEA), according to indications, contributes to the patient’s well-being.

Medicines should be increased in situations of stress, infection, surgery or injury, and should be used for life. However, unnecessarily high doses and for a prolonged period can lead to osteoporosis (fragile and brittle bones), insomnia and mood swings.

Home care

In emergency cases, such as adrenal crisis, one way out is to have an injection of hydrocortisone available, which can be applied to the muscle or vein. Other alternatives include injections of salt, fluids and other glucocorticoid hormones.

The doctor can give instructions for use, but in situations where the patient is not in a position, family members can do it. It is worth mentioning that the procedure only stabilizes the condition, it does not replace medical treatment . Subsequently, the health professional should be consulted.

Alternative therapies

As stress intensifies the symptoms of the disorder, some alternatives, such as yoga and meditation, can help relieve it:

Yoga

The word yoga comes from the Sanskrit, Indian language and that means “to unite or to integrate”. The aim is to work on the individual’s physical and psychological conditioning, through meditation, breathing techniques and postures.

Meditation

Practicing meditation consists of focusing on an object, thought or some specific activity, in order to have a greater mental and emotional understanding.

Medicines

The drugs commonly used to stabilize Addison’s disease are:

• Fludrocortisone Acetate ( Florinefe );
• Hydrocortisone ( Cortison );
• Prednisona ( Artinizona , Ciclorten , Corticorten , Crispred , Meticorten , Predinis ).

Attention!

NEVER self-medicate or stop using a medication without first consulting a doctor. Only he will be able to tell which medication, dosage and duration of treatment is the most suitable for his specific case. The information contained in this website is only intended to inform, not in any way intended to replace the guidance of a specialist or serve as a recommendation for any type of treatment. Always follow the instructions on the package insert and, if symptoms persist, seek medical or pharmaceutical advice.

Living together

People affected by primary adrenal insufficiency and who use the medication, do not suffer restrictions in their normal diet or in their daily chores. However, episodes of fatigue are recurrent.

Failure to take one of the doses or indiscipline about the times determined by the doctor can result in exhaustion or insomnia. Failure to administer it completely can cause an adrenal crisis.

Some people may experience related illnesses, such as diabetes or hypothyroidism , which requires other treatment measures.

The consultation with the specialist should take place from 6 to 12 months, with the purpose of monitoring the patient’s clinical status, being able to change the medication dose, if necessary.

Certain precautions help to avoid setbacks:

• Updated prescriptions for medicines for continuous use;
• Reserve medications available as well as the injection kit;
• Take the pills at the appointed time;
• Warn family and friends about the condition and what they should do in situations of Addisonian crisis.

Emergency identification

For patients with Addison’s disease, it is essential to inform their condition through a bracelet, card or necklace and leave medicines and dosage registered.

In serious accidents, healthy individuals release a greater amount of cortisol, which eases the stress and tension caused in the body. For people who do not produce it, the injection of hydrocortisone is necessary, as it acts to replace cortisol, preventing an adrenal crisis.

In attendance, doctors will need to know, in addition to their condition, the medications and the correct dosage that the patient will need to receive.

Medical alert bracelets or necklaces contain information about the disease and an emergency contact number. They can be found at retailers.

Complications

If Addison’s disease is not treated, it can progress to an adrenal crisis and lead to serious complications such as shock, seizures , coma or even sudden death.

State of shock

In shock, the oxygen flow slows down or stops completely. As a result, the functioning of the organs or tissues is compromised, which can cause multiple organ failure and other lethal complications.

In this condition, the person has a fast, weak or nonexistent pulse, dilated pupils, loss of consciousness, among other symptoms.

Convulsions

Seizures arise from changes in the brain’s electrical activities. Symptoms may be noticeable or not, lasting for seconds or up to 15 minutes. In severe convulsions, intense tremors and loss of control can occur, in some cases, culminating in injuries.

Coma

Coma comes from the Greek word “koma” and it means “deep sleep”. The individual loses consciousness for a period of 4 weeks and progressively improves, but there are cases of patients who have remained in a coma for years or decades.

This conditioning is generated by a momentary or permanent injury to the brain. In addition to unconsciousness, the person cannot wake up, does not react to stimuli, such as pain, sound and light.

Bodily activities such as breathing and blood circulation are not impaired. However, healthcare professionals need to ensure that the patient is healthy, safeguarding their life and brain functioning.

How to prevent

The appearance of primary adrenal insufficiency can be prevented only by preventing diseases and infections that originate the disorder. Autoimmune conditions cannot be avoided.

Those with the disorder, on the other hand, should pay attention to the necessary care to prevent acute crises that can result in their death.

Addison’s disease in dogs

As it is a non-infectious or transmissible disease, there is no risk if the dog approaches other animals or humans.

In general, the symptoms are similar to that of men:

• Pain in the abdominal region;
• Lack of appetite;
• Diarrhea;
• Vomiting;
• Drink a lot of water;
• Urinate a lot;
• Dehydration;
• Loss of body mass;
• Loss of hair;
• Skin sensitivity;
• Apathy.

Primary adrenal insufficiency can be confused with other diseases, which can lead to incorrect treatment, leading to the death of your best friend.

However, presenting such indications does not mean that the animal has Addison’s disease. Therefore, the veterinarian should be called to see what is the cause of the problem.

Addison’s disease is a rare condition, caused by autoimmune diseases or other pathologies. It has no cure, but with proper treatment, the individual can lead a normal life.

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