Primary Adrenal Insufficiency (AI), also known as Addison's Disease, is characterized by partial or complete decrease in adrenal gland hormone production , either by cancer that spreads to the adrenal glands, either by surgical removal, tuberculosis, bleeding or disease autoimmune, the latter being the most common.

Thomas Addison (doctor) first described, 150 years ago, a clinical salt loss syndrome in individuals with cutaneous hyperpigmentation, associated with the destruction of the adrenal glands.

The symptoms of adrenal insufficiency (AI) usually develop over months or years. It is estimated that the signs are only visible when 90% of the gland has been affected. This disease is fatal if left untreated, as it is accompanied by hypotension and / or hypovolemia.

 

After the development of serum antibodies to the key steroidogenic enzyme, 21-hydroxylase, patients have a period of compensated or preclinical disease, characterized by elevations in adrenocorticotropic hormone (ACTH) and renin, before symptomatic AI appears. The etiology of autoimmune primary AI has a strong genetic component in humans and several breeds of dogs are also susceptible.

Addison's disease develops in about 9-14 cases per 100,000 inhabitants annually. It occurs in all age groups, mainly women around 40 years of age, and tends to become clinically apparent during metabolic stress or trauma.

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Signs and symptoms

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Weakness, fatigue and orthostatic hypotension are early signs and symptoms of Addison's disease.

Hyperpigmentation is characterized by diffuse tanning of exposed areas of the body and, to a lesser extent, of unexposed areas, especially at pressure points (bony prominences), skin folds, scars and extensor surfaces. Black freckles are common on the forehead, face, neck and shoulders. Black-blue pigmentation occurs in the areolas and mucous membranes of the lips, mouth, rectum and vagina.

 

In general, there is anorexia, nausea, vomiting and diarrhea. Decreased cold tolerance and hypometabolism can be observed. Dizziness and syncope may occur.

The gradual onset and the non-specific nature of the symptoms often lead to the incorrect diagnosis of neurosis. Weight loss, dehydration and hypotension are characteristics of the advanced stages of Addison's disease

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Main symptoms: Fever, refractory hypotension (nausea and vomiting), refractory hypoxemia (hypoglycemia and or hypertension), Eosinophilia in critically ill patients (hypercalcemia and or metabolic acidosis).

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Diagnosis

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The diagnosis of Addison's disease is usually made in more advanced stages of the disease, because the wear and tear of the adrenal glands most often occurs more slowly and the symptoms usually appear one by one, making diagnosis difficult.

 

The doctor's specialty is endocrinology, first he will do clinical examinations and learn about his history and the evolution of his disease, he will request laboratory and imaging tests. The requested blood test aims to check the concentration of sodium and potassium in the blood, in addition, the ACTH stimulation test can be performed, in which the cortisol concentration is measured before and after the application of the synthetic ACTH injection.

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This measures the ability of your adrenal glands to produce cortisol and involves taking two blood samples. The first is a 'baseline' reading to measure the amount of cortisol in the blood when at rest. You are given an injection of a hormone called ACTH (a hormone normally produced by the pituitary gland to instruct the adrenal glands on what to do) and a second blood sample is collected after 30 to 60 minutes. If the adrenal glands are healthy, the production of cortisol in the second sample will exceed a certain level, usually 500-550 nmol / L. On the other hand, adrenal glands that are not functioning properly will not be able to produce that amount of cortisol.

Addison's disease is diagnosed by demonstrating the inability of exogenous ACTH to raise plasma cortisol.

Other blood tests requested will be aldosterone, renin, sodium and potassium.

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• Electrolytes

• Serum cortisol

• Serum ACTH

• Sometimes ACTH stimulation test

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Clinical signs and symptoms suggest adrenal insufficiency. Sometimes the diagnosis is only considered in the discovery of characteristic abnormalities of serum electrolytes, including low sodium (<135 mEq / L), high potassium (> 5 mEq / L), low HCO3 (15 to 20 mEq / L) and urea elevated

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A new blood test with antibodies is recommended to determine the cause of adrenal insufficiency. If this is negative, an adrenal scan and other tests may be necessary. Regarding image exams, ultrasound, computed tomography and magnetic resonance imaging can be requested to assess the adrenal glands.

In a person with Addison's disease, cortisol will always be low, sodium low and potassium increased and ACTH increased.

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Results that suggest Addison's disease.

Confirmatory serum test for Addison's disease

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Treatment

 

• Hydrocortisone or prednisone or p rednisolone or dexamethasone.

• Fludrocortisone

• Dose increase during concomitant illness

People with Addison's Disease will need to take daily medication to keep their bodies functioning well for the rest of their lives. These drugs replace hormones that your adrenal glands can no longer produce.

Most people take the medication three times a day, when they wake up, and then five to six hours a day during the day. The amount and type of medication varies greatly from patient to patient and depends on the type of adrenal insufficiency in which they were diagnosed.

Our body controls the levels of corticosteroids according to your need (we need to learn to be aware of our body's signals). Levels tend to be much higher in the early morning than at the end of the day, night doses should generally be avoided as they can cause insomnia.

Some people use prednisolone or prednisone or dexamethasone. The easiest way to adjust dosages is to make sure that renin concentrations are at normal doses. Normal hydration and the absence of orthostatic hypotension are evidence of adequate fluid replacement.

There are addisonians who are classified as salt losers and also need to take fludrocortisone tablets to supply aldosterone and it is usually taken in one or two doses daily to help restore the body's normal excretion of sodium and potassium.

 

In some patients, fludrocortisone causes hypertension, which is treated by reducing the dosage or initiating a non-diuretic antihypertensive. Some doctors tend to administer little fludrocortisone to avoid using antihypertensives.

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Adrenal Crisis

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About 8% of people with Addison's disease suffer from crisis adrenal gland each year, some people more often than others. It is vitally important that you learn the signs of a impending crisis and what to do in this medical emergency.

An adrenal crisis occurs when the cortisol present in your body it is not enough to keep it in perfect working order. A situation from stress, another illness or accident can worsen adrenal insufficiency and cause an adrenal crisis However, the most common cause is the abrupt discontinuation of the use of exogenous corticosteroids. Symptoms may include: Brown discoloration on the tongue and teeth, sudden, penetrating pain in the legs, lower back or abdomen, severe vomiting and diarrhea, resulting in dehydration, hypotension that can be severe, leading to hemodynamic shock, coma, hypoglycemia, loss of memory, the weakness progresses until the patient feels continually fatigued, needing bed rest and his voice may fail, so that the speech finally becomes inaudible and indistinct.

 

If this happens to you or someone you are caring for, run to the emergency room, take your letter and your card and say that it is an addisonian crisis and that you need serum and hydrocortisone.

 

Seek immediate medical attention:

• Stabilize the patient with normal saline (intravenously) and inject hydrocortisone.

• Keep the patient lying down - they can become profoundly hypotensive when sitting.

• Get them to a hospital urgently.

• Take your letter with the dosage of the emergency dose.

Note: lower doses of hydrocortisone / saline infusion rate are appropriate for children.