Addison's disease, also called adrenal insufficiency, is a rare disease that occurs when the body cannot produce enough of certain hormones. Addison's disease occurs due to the inability of the adrenal glands, located just above the kidneys, to secrete cortisol adequately. Addison's disease is not a sex-linked disease and can occur in all age groups.
What Are the Causes of Addison's Disease?
Addison's disease is a disease that develops due to damage to the adrenal glands and as a result, the body does not produce enough cortisol and aldosterone. Part of the endocrine system, the adrenal glands produce hormones that keep nearly every organ and tissue in the body functioning properly.
The adrenal glands consist of two parts. The inner part, called the medulla, produces adrenaline-like hormones. The outer layer, called the cortex, provides the secretion of hormones called corticosteroids. Corticosteroids also include the following hormones:
Glucocorticoids: These hormones, which contain cortisol, enable the body to convert food into energy and also help the body respond to situations such as stress.
• Mineralocorticoids: These hormones, which contain aldosterone, maintain the sodium and potassium balance in the body in order to keep blood pressure at normal levels.
Androgens: Androgens are the name given to the hormones secreted in the shell of the adrenal gland in both men and women. Insulin sensitivity in metabolism is effective in determining the body's fat and muscle tissue and in the distribution of these tissues.
Addison's disease is divided into two groups. The first is primary adrenal insufficiency. The other group is called secondary adrenal insufficiency.
Primary adrenal insufficiency is usually the result of damage to the cortices. This condition is often associated with immune system problems.
Other causes of adrenal gland failure are as follows:
• Tuberculosis (TB)
Other infections in the adrenal glands
Spread of cancer to the adrenal glands
Bleeding in the adrenal glands
Secondary adrenal insufficiency occurs due to insufficient production of the pituitary hormone ACTH, also called adrenocorticotropic. Many of the symptoms seen in secondary adrenal insufficiency are similar to those seen in primary adrenal insufficiency. However, people with secondary adrenal insufficiency do not have skin darkening (hyperpigmentation) and are less likely to experience severe dehydration or low blood pressure. However, these patients are more likely to experience low blood sugar.
What Are the Symptoms of Addison's Disease?
The symptoms of Addison's disease usually develop slowly over several months. Often, the disease progresses so slowly that symptoms may not be noticed until something like an illness or injury occurs.
Common symptoms of Addison's disease can be listed as follows:
•Overstrain
Weight loss and decreased appetite
• Darkening of the skin (hyperpigmentation)
Low blood pressure Fainting
Increase in salt consumption
Low blood sugar (hypoglycemia)
Nausea, diarrhea or vomiting (gastrointestinal symptoms)
•Stomach ache
Muscle or joint pain
•Irritability
•Depression
• Loss of body hair or sexual dysfunctions in women
Acute adrenal insufficiency (Addison's Crisis)
In some cases, the symptoms of Addison's disease may appear within a very short period of time. This condition, called acute adrenal insufficiency or Addison's crisis, can be life-threatening. For this reason, it is useful to apply to the hospital urgently in cases where the following symptoms are observed:
Severe dehydration
• Cooling of the skin
• Pale and moist skin
•Sweating
• Rapid breathing
• Dizziness
• Severe vomiting or diarrhea
• Weak and weak muscles
•Headache
• Drowsiness or loss of consciousness
Along with the Addisonian crisis, the following symptoms may also occur:
•Hypotension
• High potassium (hyperkalemia) and low sodium (hyponatremia)
In the hospital, a vascular access is made in the arm to relieve dehydration and the patient is given plenty of fluids. This liquid contains a mixture of salt and sugar (sodium, glucose and dextrose) that the body lacks at that time. In order to replace the missing cortisol hormone, the patient is injected with hydrocortisone.
When to Seek Medical Help for Addison's Disease?
In cases where the following symptoms are observed in Addison's disease, it is of great importance to consult a specialist doctor immediately:
• Darkening areas on the skin (hyperpigmentation)
• Feeling of tiredness
• Sudden weight loss
• Stomach and intestinal problems such as nausea, vomiting and abdominal pain
• Dizziness or fainting
• The desire to consume too much salt
•Muscle and joint pain
How Is Addison's Disease Diagnosed?
In order to diagnose Addison's disease, the doctor first asks the patient some questions about the medical history and symptoms. As a result of this evaluation, if deemed necessary, a few of the following tests may be requested from the patient:
• Blood analysis: In the blood test, the sodium, potassium, cortisol and adrenocorticotropic hormone (ACTH) levels in the blood, which enable the production of adrenal cortex hormones, are measured. In addition, antibodies associated with Addison's disease found in the immune system can also be examined in the blood test.
• ACTH stimulation test: ACTH causes the adrenal glands to produce cortisol. This test detects the level of cortisol in the blood before and after an injection of synthetic ACTH.
• Insulin-induced hypoglycemia test: In cases where the doctor thinks that the patient has adrenal insufficiency as a result of pituitary disease (secondary adrenal insufficiency), the patient may be asked to have this test performed. This test measures how high the blood sugar and cortisol levels are after an injection of insulin. In healthy people, after this test, the glucose level drops, while the cortisol level rises.
• Imaging tests: A computed tomography (CT) scan of the abdomen may be used to check the size of the adrenal glands and to understand other abnormalities. If secondary adrenal insufficiency is detected as a result of the test, the pituitary gland should also undergo an MRI scan.
How Is Addison's Disease Treated?
Medication is used in the treatment of Addison's disease. In order to increase the levels of steroid hormones that the body cannot produce to normal levels, the patient should regularly take drugs containing hormones. Some treatment options may include oral corticosteroids:
• Hydrocortisone, prednisone or methylprednisolone hormones to replace cortisol are given to the patient in a way that mimics the normal 24-hour fluctuation of cortisol level.
• Fludrocortisone acetate to replace aldosterone. Especially during heavy exercise, when the weather is hot, or when the patient has gastrointestinal disorders such as diarrhea, plenty of salt should be used in meals. In addition, in cases where the body is under stress due to surgery, infection or a mild illness, a temporary increase in the dose of medication used under the supervision of a doctor may be required. In cases where patients complain of vomiting, corticosteroid injections can be applied.
Living with Addison's Disease
Suggestions for living with Addison's disease are as follows:
•Patients should always carry a medical alert card and wristband. The steroid emergency card allows healthcare professionals to understand what kind of care a patient needs.
•Patients should keep their medicines in a place where they can reach them easily. Failure to take medications, even for one day, can cause serious complications. For this reason, it is very important for patients to have a small amount of medication with them at work or while traveling.
•Patients should also always carry a glucocorticoid injection kit with them. This kit contains a syringe and an injectable corticosteroid for use in an emergency.
•Patients should always be in contact with their doctor. It is very important to be in constant communication with the doctor to make sure that the hormone doses taken are adequate and not overdone. In cases where there are ongoing problems with medications, the dose or timing of medication may need to be changed. In this regard, the doctor will support the patients by providing the necessary information.
• Going to the annual controls without interruption is a critical issue in terms of keeping the course of this disease under control. For this reason, it is beneficial for patients to see their own doctor or an endocrinologist at least once a year.