Last Updated: 27th December, 2020
Addison’s Disease is a rare condition that happens when the body does not contain enough of inevitable hormones, often termed adrenal insufficiency. In Addison’s disease, far less cortisol and sometimes too scant aldosterone are developed by the adrenal glands located just above your kidneys.
Across both age groups and all genders, Addison’s disease exists and might even be life-threatening. To replace those that are suffering from a lack, therapy includes taking hormones.
Causes Of Addison’s Disease
Addison’s disease is aggravated damage to the adrenal glands, resulting in inadequate cortisol hormones and, also, insufficient aldosterone. In your endocrine system, your adrenal glands are part of it. They generate hormones that provide direction to almost every organ and tissues of the body.
Your adrenal glands consist of two parts. The interior (medulla) emits hormones resembling adrenaline. A group of hormones that regulate corticosteroids are formed by the outer layer (cortex). Among the corticosteroids are:
- Glucocorticoids: These hormones, which include cortisol, influence your body’s ability to convert food into energy, play a role in your immune system’s inflammatory response and restore your body respond to stress.
- Mineralocorticoids: These hormones, which include aldosterone, maintain your body’s balance of sodium and potassium to keep your blood pressure normal.
- Androgens: These male sex hormones are produced in limited amounts by the adrenal glands in both men and women. They cause reproductive development in men, and influence muscle mass, sex drive (libido) and a sense of well-being in both men and women.
Primary Adrenal Insufficiency
When the cortex is damaged and doesn’t produce enough adrenocortical hormones, the condition is considered primary adrenal insufficiency. This is most commonly the result of the body attacking itself (autoimmune disease). For unknown reasons, your immune system views the adrenal cortex as foreign, something to strike and eliminate. People with Addison’s disease are more likely than others to acquire another autoimmune disease as well.
Other causes of adrenal gland failure may include:
- Other infections of the adrenal glands
- Spread of cancer to the adrenal glands
- Bleeding into the adrenal glands. In this case, you may have an Addisonian crisis without any previous symptoms.
Secondary Adrenal Insufficiency
The pituitary gland makes a hormone called Adrenocorticotropic Hormone (ACTH). ACTH, in turn, stimulates the adrenal cortex to produce its hormones. Benign pituitary tumors, inflammation and prior pituitary surgery are common causes of not producing enough pituitary hormones.
Too little ACTH can lead to too little of the glucocorticoids and androgens normally produced by your adrenal glands, even though your adrenal glands themselves aren’t damaged. This is called secondary adrenal insufficiency. Mineralocorticoid production is not affected by too little ACTH.
Most symptoms of secondary adrenal insufficiency are similar to those of primary adrenal insufficiency. However, people with secondary adrenal insufficiency don’t have hyperpigmentation and are less likely to have severe dehydration or low blood pressure. They’re more likely to have low blood sugar.
A temporary cause of secondary adrenal insufficiency occurs when people who take corticosteroids (for example, prednisone) to treat chronic conditions, like asthma or arthritis, stop taking the corticosteroids all at once rather than tapering off.
Addison’s Disease Symptoms
Addison’s disease symptoms usually develop slowly, often over several months. Often, the disease progresses so slowly that symptoms are ignored until a stress, such as illness or injury, occurs and makes symptoms worse. Signs and symptoms may include:
- Severe fatigue
- Weight loss and decreased appetite
- Darkening of your skin (hyperpigmentation)
- Low blood pressure, even fainting
- Salt craving
- Low blood sugar (hypoglycemia)
- Nausea, diarrhea or vomiting (gastrointestinal symptoms)
- Abdominal pain
- Muscle or joint pains
- Depression or other behavioral symptoms
- Body hair loss or sexual dysfunction in women
Acute Adrenal Failure (Addisonian Crisis)
Every so often the signs and symptoms of Addison’s disease may appear suddenly. Acute adrenal failure (Addisonian crisis) can lead to fatal shock. Seek emergency medical treatment if you experience the following signs and symptoms:
- Severe weakness
- Pain in your lower back or legs
- Severe abdominal pain, vomiting and diarrhea, leading to dehydration
- Reduced consciousness or delirium
In an Addisonian crisis you will also have:
- Low blood pressure
- High potassium (hyperkalemia) and low sodium (hyponatremia)
Addison’s Disease Complications
If you have an untreated Addison’s disease, you may develop an Addisonian crisis as a result of physical stress, such as an injury, infection or illness. Normally, the adrenal glands produce two to three times the usual amount of cortisol in response to physical stress. With adrenal insufficiency, the inability to increase cortisol production with stress can lead to an Addisonian crisis.
An Addisonian crisis is a life-threatening situation that results in low blood pressure, low blood levels of sugar and high blood levels of potassium. You will need immediate medical care. People with Addison’s disease commonly have associated autoimmune diseases.
Diagnosis Of Addison’s Disease
Your doctor will talk to you first about your medical history and your signs and symptoms. You may undergo some of the following tests:
- Blood Test: Tests can measure your blood levels of sodium, potassium, cortisol and Adrenocorticotropic Hormone (ACTH), which stimulates the adrenal cortex to produce its hormones. A blood test can also measure antibodies associated with autoimmune Addison’s disease.
- ACTH Stimulation Test: ACTH signals your adrenal glands to produce cortisol. This test measures the level of cortisol in your blood before and after an injection of synthetic ACTH.
- Insulin-Induced Hypoglycemia Test: You may be assigned this test if doctors think you may have adrenal insufficiency as a result of pituitary disease (secondary adrenal insufficiency). The test involves checking your blood sugar (blood glucose) and cortisol levels after an injection of insulin. In healthy people, glucose levels fall and cortisol levels increase.
In certain situations, doctors may undergo alternative tests for secondary adrenal insufficiency, such as a low-dose ACTH stimulation test, prolonged ACTH stimulation test or glucagon stimulation test.
- Imaging Tests: You may undergo a computerized tomography (CT) scan of your abdomen to check the size of your adrenal glands and look for other abnormalities. You may also undergo an MRI scan of your pituitary gland if testing indicates you might have secondary adrenal insufficiency.
Addison’s Disease Treatment
All treatment for Addison’s disease involves medication. You will be issued hormone replacement therapy to correct the levels of steroid hormones your body isn’t producing. Some options for treatment include oral corticosteroids such as:
- Hydrocortisone (Cortef), prednisone or methylprednisolone to replace cortisol. These hormones are given on a schedule to mimic the normal 24-hour fluctuation cortisol levels.
- Fludrocortisone acetate to replace aldosterone.
You will need to get plenty of salt (sodium) in your diet, especially during heavy exercise, when the weather is hot or if you have gastrointestinal upsets, such as diarrhea.
Your doctor will also suggest a temporary increase in your medication dosage if your body is stressed, like from an operation, an infection or a minor illness. If you’re ill with vomiting and can’t keep down oral medications, you may need injections of corticosteroids.
Other treatment recommendations include:
- Carry a medical alert card and bracelet at all times. A steroid emergency card and medical alert identification will let emergency medical personnel know what kind of care you need. In addition, have a written action plan.
- Keep extra medication handy. Pining for even one day of medication may be dangerous, therefore, maintain a scant supply of medication at work and with you whenever you travel.
- Carry a glucocorticoid injection kit. The kit contains a needle, syringe and injectable forms of corticosteroids to use in case of emergency.
- Stay in contact with your doctor. Keep an ongoing relationship with your doctor to make sure the doses of replacement hormones are adequate but not excessive. If you’re having ongoing problems with your medications, you may need adjustments in the doses or the timing of the medications.
- Have annual checkups. See your doctor or an endocrinology specialist at least once a year. Your doctor may recommend annual screening for a number of autoimmune diseases.
Treatment in an Addisonian crisis, which constitutes a medical emergency, typically includes intravenous injections of:
- Saline solution
- Sugar (dextrose)
Addison’s Disease Prevention
Addison’s disease can’t be prevented, but there are steps you can secure to prevent an Addisonian crisis:
- Talk to your doctor if you always feel tired, weak or are losing weight. Ask about experiencing an adrenal shortage.
- If you have been diagnosed with Addison’s disease, seek your doctor about what to do when you’re ill. You may need to learn how to increase your dose of corticosteroids.
- If you become terribly weary, especially if you are vomiting and can’t obtain your medication, go to the emergency room.
Some people with Addison’s disease worry about serious side effects from hydrocortisone or prednisone because they recognize these occur in people who receive these steroids for other reasons.
Nevertheless, the side effects of high-dose glucocorticoids do not happen if you have acquired Addison’s disease, since the dose you are given supplements the quantity that is lacking. Be sure to follow up on a regular basis with your doctor to ensure your dosage might not be abnormally high.