Addison's disease, also known as primary adrenal insufficiency and hypocartisolism, is a rare condition in which the adrenal glands do not make enough of the hormones cortisol and sometimes aldosterone.
Symptoms tend to come and go and can include stomach pain, dizziness, fatigue, weight loss, salt cravings, and darkening of the skin. During times of extreme stress or poor health, Addison's disease can cause a rapid drop in cortisol levels and a life-threatening event known as an adrenal crisis .
The most common cause of Addison's disease is autoimmune adrenalitis, a disease in which the body's immune system inadvertently attacks and damages adrenal cells.
Addison's disease can be diagnosed through blood tests and imaging, but it is so rare that it only affects one person in 100,000. – that the diagnosis is usually postponed until more serious manifestations of the disease develop.
Addison's disease cannot be cured, but it can be significantly improved with hormone replacement therapy and prevention of common triggers. With proper treatment, Addison's disease can be controlled and you can be sure that you will live a long and healthy life.
Symptoms of Addison's disease
The symptoms of Addison's disease are directly related to the degree of adrenal involvement. As a progressive disease, symptoms tend to get worse over time if left untreated.
They can start out randomly, with strange moments of abdominal pain and weakness, but they become more apparent as the attacks recur, especially during times of stress.
Depending on the cause of the underlying disorder, Addison's disease symptoms can develop slowly and steadily or quickly and violently.
Most symptoms occur when a drop in cortisol levels (hypocartisolism) causes an associated drop in blood pressure ( hypotension ) and blood sugar ( hypoglycemia ). Other symptoms are associated with a drop in the levels of aldosterone, the hormone responsible for sodium retention.
The main symptoms of Addison's disease tend to be episodic and nonspecific. Common symptoms include:
- Abdominal pain
- Decreased appetite
- Salt craving
- Dizziness when standing up ( postural hypotension )
- Blurry vision
- Shaking or shaking
- Nausea or vomiting
- Anxiety or irritability
- Needle sensation ( peripheral neuropathy )
- Decreased sex drive (low libido)
- Hair loss
- Muscle or joint pain
- Darkening of the skin (hyperpigmentation), especially in areas with folds or scars on the skin, as well as on the palms, nipples, and inside of the cheeks.
An adrenal crisis, also known as an Addison crisis, occurs when cortisol levels drop so rapidly that it causes severe and life-threatening symptoms, including:
- Severe vomiting and / or diarrhea causing dehydration.
- Pain in the lower back, legs, or abdomen.
- Fainting ( fainting )
- Muscle spasms
- Confusion and disorientation
- Intense thirst and inability to urinate.
- Abnormal heart beat ( arrhythmia )
- Periodic muscle paralysis
Death can occur if not treated aggressively with intravenous fluids and steroids, most often as a result of hypotensive shock or respiratory failure . According to research, adrenal crisis leads to death in one in 16 cases, most of the time due to inopportune or inadequate treatment.
Addison's disease is caused by failure of the adrenal glands. The adrenal glands are one of several organs that make up the endocrine system . The glands are located above each kidney and are responsible for the production of various hormones, such as cortisol, aldosterone, and adrenaline.
Cortisol, often known as the "stress hormone", triggers the fight or flight instinct during a crisis, but it also plays a central role in regulating blood pressure, blood sugar, inflammation, sleep and energy levels. It occurs in a part of the adrenal gland known as the zona fascial .
Aldosterone helps regulate sodium and potassium balance in the body and thus affects blood pressure, blood volume, and water retention in cells. It occurs in another part of the gland known as the zona glomerulosa .
Addison's disease develops when the adrenal glands can no longer supply enough cortisol and aldosterone to the body. It affects men and women equally and occurs in all age groups, but is most common between the ages of 30 and 50.
There are several reasons why the adrenal glands stop working properly. In Addison's disease, primary adrenal insufficiency is usually caused by a disease or disorder that directly affects the organ itself.
The most common cause of this is autoimmune adrenalitis, an autoimmune disease in which the body shifts its defenses towards itself and attacks the steroid-producing adrenal cells. No one knows exactly why this happens, although genetics are believed to play a central role.
Generally speaking, the zona fascial (where cortisol is produced) is the site of the main autoimmune attack, followed by the zona glomerular (where aldosterone is produced). In some cases, the third and final zone, known as the reticular zone , may have accompanying damage, leading to a disruption in the production of certain sex hormones.
In addition to autoimmune adrenalitis, other conditions can directly affect the adrenal glands, including:
- Common HIV infection
- Systemic fungal infections
- Secondary ( metastatic ) cancer
- Adrenal hemorrhage
- Congenital adrenal hyperplasia , a genetic disorder characterized by abnormal enlargement of the adrenal glands.
- Adrenomyeloneuropathy , an inherited neurological disorder that can cause adrenal gland failure (usually in adulthood).
Less commonly, a genetic disorder can indirectly affect adrenal function by depriving the body of compounds necessary to make steroids. One of those compounds is cholesterol, which the adrenal glands convert to cortisol and aldosterone.
Rare genetic disorders such as Smith-Lemli-Opitz syndrome (a congenital error that affects cholesterol synthesis) and abetalipoproteinemia (which interferes with fat absorption) are some of the inherited diseases that can indirectly lead to cholesterol disease. Addison.
An adrenal crisis can occur as a continuation of Addison's disease or independently of it. Specifically, an adrenal crisis develops when the drop in cortisol levels is rapid and severe, resulting in low blood pressure, low blood sugar, and high blood potassium levels. The condition is life threatening and requires immediate medical attention.
An adrenal crisis can be caused by any event in which the body cannot respond adequately to extreme psychological or physical stress. According to one study, the most common triggers for adrenal crisis are as follows.
Other studies have also confirmed that gastrointestinal diseases are the main trigger for acute adrenal crisis. Other causes include seizures, severe migraines, hot weather, atrial fibrillation (irregular heartbeat), and even long-haul flights. Additional risk factors include pregnancy and diabetes.
Secondary adrenal insufficiency
An adrenal gland crisis can also occur when the pituitary gland is damaged or diseased. The pituitary gland is part of the hypothalamic-pituitary-adrenal axis. Its job is to "instruct" the adrenal glands when and how much to make more cortisol. It does this with the help of adrenocorticotropic hormone (ACTH).
If the crisis occurs as a result of pituitary insufficiency (possibly caused by cancer or surgery on the pituitary gland), it is called secondary adrenal insufficiency.
Another common cause of secondary adrenal insufficiency is the sudden cessation of corticosteroid medications used to treat arthritis, asthma, and other inflammatory conditions. When medications are abruptly stopped, undiagnosed Addison's disease can be inadvertently "exposed" in the form of an adrenal crisis.
Addison's disease is diagnosed by various blood tests and imaging tests that are used to confirm adrenal insufficiency. The diagnosis is usually based on the appearance of characteristic symptoms such as hyperpigmentation, fatigue, and salt cravings (the classic adrenal triad).
In other cases, routine blood tests may reveal abnormalities that indicate disease, such as unexplained recurrent episodes of hypoglycemia, hyponatremia, and hypokalemia.
Very often, an adrenal crisis becomes the first symptom of Addison's disease in about 50% of cases.
Tests used to diagnose Addison's disease
- Serum cortisol tests
- ACTH stimulation test
- Investigation of autoantibodies
- Insulin-induced hypoglycemia test
- Visual tests
- Serum cortisol tests can be used to measure cortisol levels in the blood. A cortisol level of 25 mcg / dl or more excludes adrenal insufficiency of any kind.
- The ACTH stimulation test involves the use of a synthetic ACTH called cosyntropin (tetracosactide). The test compares cortisol levels before and after an ACTH injection. A cortisol level of 7 mcg / dl or more at baseline, rising to 20 mcg / dl or more in 30-60 minutes, excludes adrenal insufficiency as a cause.
- Autoantibody studies can be used to detect proteins called 21-hydroxylase antibodies that are associated with autoimmune adrenalitis.
- The insulin-induced hypoglycemia test is used to diagnose secondary adrenal insufficiency. The test compares blood glucose and cortisol levels before and after an insulin injection to see if the pituitary gland responds correctly.
- Imaging tests such as ultrasound, computed tomography (CT) , and magnetic resonance imaging (MRI) will be ordered to check for abnormalities in the adrenal glands themselves.
Additional tests may be required to rule out all other causes of adrenal insufficiency, a process known as differential diagnosis . Other possible causes include hyperthyroidism (overactive thyroid gland), lymphoma , anorexia nervosa, hemochromatosis (iron overload), and adrenal suppression due to corticosteroid therapy.
Addison's disease, as a disorder characterized by the depletion of key hormones, is primarily treated and treated with hormone replacement therapy throughout life. In most cases, this will involve taking oral steroids two to three times a day.
Among the most prescribed:
- Cortef (hydrocortisone) is used to replace cortisol. The drug is prescribed in a daily dose of 15 to 25 milligrams and is taken in two or three doses with meals and washed with a full glass of water to prevent indigestion.
- Florinef (fludrocortisone) is used to replace aldosterone. It is prescribed in a daily dose of 50 to 200 mcg in one or two doses with or without food.
- Dehydroepiandrosterone (DHEA) is an oral steroid supplement that is sometimes prescribed to relieve chronic fatigue associated with Addison's disease, especially in women. It is prescribed in a daily dose of 25 to 50 milligrams when you are awake or before bed.
- Prednisone , another widely used corticosteroid, can stimulate cortisol production and can be used if you cannot tolerate cortef. Prednisone can be taken in a daily dose of 3 to 5 milligrams.
- Dexamethasone is an injectable corticosteroid that can be used to treat intolerance to cortef. It is administered intramuscularly at a daily dose of 0.5 milligrams.
As with many medications, those commonly prescribed to treat Addison's disease have several common side effects, including:
- Stomach ache
- Dry Skin
- Slight bruising
- Muscular weakness
- Delayed wound healing
- Menstrual changes
Long-term use can cause lipodystrophy (redistribution of body fat) and osteoporosis (thinning of the bones), although this is more likely due to drug overdose than duration of treatment.
Grapefruit and licorice can also increase side effects, so they should be used with caution. Talk to your doctor if the side effects are unbearable or worrisome. In some cases, you can change the treatment or reduce the dose.
If you have Addison's disease, you will probably be advised to wear a medical identification bracelet in case of an adrenal crisis. Also, you will probably be advised to bring an emergency kit that contains a needle, a syringe, and a 100-milligram ampoule of hydrocortisone for injection.
In case of vomiting, persistent diarrhea, or other symptoms of an adrenal crisis, seek emergency help immediately.
Because an adrenal crisis can progress rapidly, you should receive an emergency intramuscular injection of hydrocortisone before help arrives. This requires that you or a member of your family learn the correct injection techniques and recognize the signs and symptoms of an adrenal crisis.
Giving too much hydrocortisone in an emergency will not be harmful. In contrast, underdosing will do little to prevent your condition from rapidly getting worse. An adrenal crisis requires hospitalization with intravenous hydrocortisone and 0.9% sodium chloride intravenous fluids.
Living with any chronic medical condition can be challenging. In Addison's disease, the biggest challenge may be preventing any disease or event that can trigger an adrenal crisis.
However, stress management is key. Particularly when you are exhausted and overwhelmed by anxiety, stress reduction techniques can reduce the risk of a seizure, or at least reduce the severity or frequency of chronic episodes.
These include techniques such as mindfulness meditation, guided imagery, progressive muscle relaxation, biofeedback, tai chi, and gentle yoga.
While exercise is still vital to good health, it should be done without causing excessive physical exertion. If you plan to do athletics, tell your doctor so the dose of medication can be increased to compensate for the increased activity. The same applies if you are sick, have a fever over 100 F, or undergo a medical procedure.
Although there is no specific diet for Addison's disease, you should avoid low sodium diets and even increase your salt intake if you plan to go outside in hot weather or exercise. If you feel tired, don't push it, give your body time to recover.
With proper medical support, women with Addison's disease can expect a healthy pregnancy and normal delivery. However, the level of the endocrine system should be controlled during the first trimester and the amount of medication should be increased as labor approaches.
If your fertility is affected, you can explore assisted reproductive technology options with a reproductive system specialist in conjunction with your endocrinologist .
Get the word of drug information
Thanks to advances in therapy, people with Addison's disease can have better health and a near-normal life expectancy. In fact, research shows that the average life expectancy for people with Addison's disease has increased to 64.8 years for men and 75.7 years for women.
Early diagnosis and treatment can further increase these rates. To this end, it's important to see your doctor if you're experiencing recurring bouts of fatigue, low blood pressure, weight loss, salt cravings, or skin discoloration that you just can't explain. An adrenal crisis does not have to be your first symptom.
If your healthcare provider doesn't know what Addison's disease is, don't hesitate to ask for a referral to an endocrinologist who can perform tests to confirm or rule out the disease.