Addison's Disease
What is Addison's disease?
Addison’s disease is a rare endocrine disorder in which the adrenal glands which is above the kidneys do not produce enough hormones like cortisol and aldosterone which control the function of tissues or organs. It affects about 1 in 100,000 people. It occurs in all age groups and afflicts men and women equally. It can be life threatening. It is most common in the 30-50 year-old age range.
Cortisol (glucocorticoids) produced helps the body respond to stress (acute illness, injury, or surgery). It also helps maintain blood pressure, heart function, the immune system, helps balance the effects of insulin in breaking down sugar and helps regulate the metabolism of proteins, carbohydrates, and fats. Cortisol is regulated by the brains hypothalamus and the pituitary gland: Hypothalamus sends releasing hormones to the pituitary gland. The pituitary then responds by secreting ACTH (adrenocorticotropin), a hormone that stimulates the adrenal glands to produce cortisol. Completing the cycle, cortisol then signals the pituitary to lower secretion of ACTH.
Aldosterone (mineralocorticoids) maintain blood pressure, water and salt balance in the body by helping the kidney retain sodium and excrete potassium. When aldosterone drops, the kidneys are not able to regulate salt and water balance, causing blood volume and blood pressure to drop.
Addison’s disease is also called “primary adrenal insufficiency" where the primary defect is at the adrenal glands. In “secondary adrenal insufficiency,”the pituitary gland does not secrete enough adrenocorticotropic hormone (ACTH), which activates the adrenal glands to produce cortisol.
Addison’s disease is a rare endocrine disorder in which the adrenal glands which is above the kidneys do not produce enough hormones like cortisol and aldosterone which control the function of tissues or organs. It affects about 1 in 100,000 people. It occurs in all age groups and afflicts men and women equally. It can be life threatening. It is most common in the 30-50 year-old age range.
Cortisol (glucocorticoids) produced helps the body respond to stress (acute illness, injury, or surgery). It also helps maintain blood pressure, heart function, the immune system, helps balance the effects of insulin in breaking down sugar and helps regulate the metabolism of proteins, carbohydrates, and fats. Cortisol is regulated by the brains hypothalamus and the pituitary gland: Hypothalamus sends releasing hormones to the pituitary gland. The pituitary then responds by secreting ACTH (adrenocorticotropin), a hormone that stimulates the adrenal glands to produce cortisol. Completing the cycle, cortisol then signals the pituitary to lower secretion of ACTH.
Aldosterone (mineralocorticoids) maintain blood pressure, water and salt balance in the body by helping the kidney retain sodium and excrete potassium. When aldosterone drops, the kidneys are not able to regulate salt and water balance, causing blood volume and blood pressure to drop.
Addison’s disease is also called “primary adrenal insufficiency" where the primary defect is at the adrenal glands. In “secondary adrenal insufficiency,”the pituitary gland does not secrete enough adrenocorticotropic hormone (ACTH), which activates the adrenal glands to produce cortisol.
Causes of Addison's disease
Addison's disease is caused by damage to the cortex of adrenal glands, resulting in not enough production of the hormones like cortisol, aldosterone and androgens. About 70 percent of reported cases of Addison's disease are due to autoimmune disorders, in which the immune system forms antibodies that attack the body's own tissues or organs and slowly destroy them. The adrenal glands are composed of medulla and cortex. Adrenal insufficiency occurs when at least 90 % of the adrenal cortex has been destroyed.
The interior medulla produces adrenaline-like hormones. The outer layer cortex produces a group of hormones:
Addison's disease is caused by damage to the cortex of adrenal glands, resulting in not enough production of the hormones like cortisol, aldosterone and androgens. About 70 percent of reported cases of Addison's disease are due to autoimmune disorders, in which the immune system forms antibodies that attack the body's own tissues or organs and slowly destroy them. The adrenal glands are composed of medulla and cortex. Adrenal insufficiency occurs when at least 90 % of the adrenal cortex has been destroyed.
The interior medulla produces adrenaline-like hormones. The outer layer cortex produces a group of hormones:
- Glucocorticoids. These hormones, which include cortisol, influence your body's ability to convert food into energy, regulate immune system's inflammatory response and help your body respond to stress.
- Mineralocorticoids. These hormones, which include aldosterone, maintain your body's balance of sodium and potassium, water to keep your blood pressure normal.
- Androgens. These male sex hormones are produced in small amounts by the adrenal glands in both men and women. They cause sexual development in men, and influence muscle mass, libido and a sense of well-being in both men and women.
Primary adrenal insufficiency
When the adrenal gland cortex is damaged and doesn't produce enough adreno-cortical hormones, the condition is called primary adrenal insufficiency. Causes for adrenal gland failure include:
When the adrenal gland cortex is damaged and doesn't produce enough adreno-cortical hormones, the condition is called primary adrenal insufficiency. Causes for adrenal gland failure include:
- Tuberculosis
- injury to adrenal glands
- Other infections of the adrenal glands
- Spread of cancer to the adrenal glands
- Bleeding into the adrenal glands
- Surgical removal of adrenal glands
- Amyloidosis
- Genetic defects
Causes of Secondary Adrenal Insufficiency
1. This is due to a lack of ACTH release from the pituitary gland, which causes a drop in the production of cortisol from the adrenal gland but not aldosterone. It can result from tumors or infections of the pituitary gland, loss of blood flow to the pituitary, radiation of pituitary tumors, or surgical removal of parts of the hypothalamus or the pituitary gland during neurosurgery.
2. Steroid consumption: A temporary form of secondary adrenal insufficiency may occur when a person who has been receiving a glucocorticoid hormone such as prednisone for a long time abruptly stops or interrupts taking the medication. Usually patients with inflammatory illnesses like rheumatoid arthritis, asthma, or ulcerative colitis take steroids.
3. Surgical removal of benign, or noncancerous, ACTH-producing tumors of the pituitary gland (Cushings disease).
1. This is due to a lack of ACTH release from the pituitary gland, which causes a drop in the production of cortisol from the adrenal gland but not aldosterone. It can result from tumors or infections of the pituitary gland, loss of blood flow to the pituitary, radiation of pituitary tumors, or surgical removal of parts of the hypothalamus or the pituitary gland during neurosurgery.
2. Steroid consumption: A temporary form of secondary adrenal insufficiency may occur when a person who has been receiving a glucocorticoid hormone such as prednisone for a long time abruptly stops or interrupts taking the medication. Usually patients with inflammatory illnesses like rheumatoid arthritis, asthma, or ulcerative colitis take steroids.
3. Surgical removal of benign, or noncancerous, ACTH-producing tumors of the pituitary gland (Cushings disease).
Symptoms of Addison's disease
Addison's disease symptoms usually develop gradually over several months. Often symptoms are ignored until a stress, such as illness or injury, occurs and makes symptoms worse. Signs and symptoms may include:
Addison's disease symptoms usually develop gradually over several months. Often symptoms are ignored until a stress, such as illness or injury, occurs and makes symptoms worse. Signs and symptoms may include:
- Extreme fatigue (more common)
- Weight loss and decreased appetite (more common)
- Muscle weakness (more common)
- Nausea, diarrhea or vomiting (gastrointestinal symptoms in 50% of patients)
- Low blood pressure especially when standing, even fainting
- Darkening of your skin (hyperpigmentation) most visible on scars; skin folds; pressure points such as the elbows, knees, knuckles, and toes; lips and mucous membranes.
- Salt craving because loss of salt
- Low blood sugar (hypoglycemia) more severe in children than adults
- Abdominal pain
- Abnormal menses
- Irritability
- Depression or other behavioral symptoms
- Body hair loss or sexual dysfunction in women
Acute adrenal failure (addisonian crisis): Medical emergency
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. Acute adrenal failure (addisonian crisis) after an injury, illness, or time of intense stress can lead to life-threatening shock. 25 %of patients, symptoms first appear during an addisonian crisis. If left untreated, it can be fatal.
Seek emergency medical treatment if you experience the following signs and symptoms:
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. Acute adrenal failure (addisonian crisis) after an injury, illness, or time of intense stress can lead to life-threatening shock. 25 %of patients, symptoms first appear during an addisonian crisis. If left untreated, it can be fatal.
Seek emergency medical treatment if you experience the following signs and symptoms:
- Severe weakness
- Confusion
- Pain in your lower back or legs
- Severe abdominal pain, vomiting and diarrhea, leading to dehydration
- Reduced consciousness or delirium
- Super low blood pressure
- High potassium (hyperkalemia) and low sodium (hyponatremia)
- Hypoglycaemia (low glucose level)
Diagnosis of Addison's disease
- Taking a thorough medical History and physical examination: Dark patches on your skin might be a clue to consider testing for Addison’s disease.
- Blood tests: These will be done to measure the levels of glucose, sodium, potassium, cortisol, and ACTH in your blood.
- ACTH stimulation test:injection of synthetic ACTH will test the adrenal glands’ response. In ACTH test, cortisol measurement in blood is repeated 30 to 60 minutes after an intravenous ACTH injection. If the adrenal glands produce low levels of cortisol after the shot, they may not be functioning properly as ACTH should stimulate cortisol production.
- Insulin-induced hypoglycemia test. If you are suspected to have secondary adrenal insufficiency as a result of pituitary disease, this test will be conducted. The test involves checking your blood glucose and cortisol levels after an injection of insulin. In healthy people, glucose levels fall and cortisol levels increase.
Alternative tests for secondary adrenal insufficiency are: low-dose ACTH stimulation test, prolonged ACTH stimulation test or glucagon stimulation test. - X-rays: Looking calcium deposits on the adrenal glands.
- Computed tomography (CT scan) or MRI: To evaluate the adrenals and/or pituitary gland.
Treatment
Patients with Addison's disease definitely needs treatment by taking medication. Treatment of Addisons disease involves replacing, or substituting, the hormones that the adrenal glands are not making. Some medication options for treatment include oral corticosteroids to replace cortisol are:
You will need to take more salt (sodium) in your diet, especially during heavy exercise, when the weather is hot or if you have gastrointestinal upsets, such as diarrhea.
You may need to temporary increase your medication dosage if your body is stressed for example from an operation, an infection or a minor illness. If you are vomiting badly or very ill such that you cant take oral medications, you may need to be hospitalized for injections of corticosteroids .
Treatment for an addisonian crisis, which is a medical emergency, typically includes intravenous injections of:
Patients with Addison's disease definitely needs treatment by taking medication. Treatment of Addisons disease involves replacing, or substituting, the hormones that the adrenal glands are not making. Some medication options for treatment include oral corticosteroids to replace cortisol are:
- Hydrocortisone, prednisone or methylprednisolone to replace cortisol. These hormones are given on a schedule to mimic the normal 24-hour fluctuation of cortisol levels.
- Fludrocortisone acetate to replace aldosterone.
You will need to take more salt (sodium) in your diet, especially during heavy exercise, when the weather is hot or if you have gastrointestinal upsets, such as diarrhea.
You may need to temporary increase your medication dosage if your body is stressed for example from an operation, an infection or a minor illness. If you are vomiting badly or very ill such that you cant take oral medications, you may need to be hospitalized for injections of corticosteroids .
Treatment for an addisonian crisis, which is a medical emergency, typically includes intravenous injections of:
- Corticosteroids
- Saline solution
- Sugar (dextrose)