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Addison's disease is a severe or total deficiency of the hormones made in the adrenal cortex, caused by a destruction of the adrenal cortex. It is a rare hormonal disorder that affects about one in 100,000 people. It occurs when the adrenal glands, located above each kidney, do not produce enough corticosteroids - in particular, the hormone cortisol, and in some cases, the hormone aldosterone.
There are normally two adrenal glands, one located above each kidney. The adrenal glands are in fact two endocrine (ductless, or hormone-producing) glands in one. The inner part of the adrenal (the 'medulla') produces epinephrine (also called adrenaline) which is produced at times of stress and helps the body respond to "fight or flight" situations by raising the pulse rate, adjusting blood flow, and raising blood sugar. However, the absence of the adrenal medulla and epinephrine does not cause disease.
In contrast, the outer portion of the adrenal - the cortex - is more critical. The adrenal cortex makes two important steroid hormones, cortisol and aldosterone. Cortisol mobilizes nutrients, modifies the body's response to inflammation, stimulates the liver to raise the blood sugar, and also helps to control the amount of water in the body. Aldosterone regulates salt and water levels which affects blood volume and blood pressure. Cortisol production is regulated by another hormone, adrenocorticotrophic hormone (ACTH), made in the pituitary gland which is located just below the brain.
Secondary adrenal insufficiency, caused by a lack of ACTH, results in a deficiency of cortisol, but usually not aldosterone. The cause is either pituitary disease, such as a tumor, or the prolonged use of "steroid" medication that suppresses ACTH. The treatment is simply to replace cortisol, usually with the synthetic steroid prednisone, but sometimes hydrocortisone or cortisone acetate.
Auto-immune Addison's disease, the most common type, can be associated with other auto-immune diseases that similarly affect other endocrine glands. The most common one is the thyroid. If an underactive thyroid (hypothyroidism) coexists with Addison's disease, this is called Schmidt's syndrome. Less commonly associated auto-immune diseases are insulin-dependent diabetes mellitus, and insufficiencies of the parathyroid glands, gonads, and vitamin B12 absorption (pernicious anemia).
Incidence; Causes & Development
There are no accurate statistics on the incidence of Addison's disease in the United States. A study in London showed 39 cases per million population as of 1960, of which 12 were due to tuberculosis. In the non-tuberculosis group, women were three times more likely to have Addison's disease. Extrapolation of these figures to the U.S. would suggest about 8,800 cases, but this is probably an underestimation.
Classical Addison's disease, also called primary adrenal insufficiency, results from a loss of both cortisol and aldosterone secretion due to the near total or total destruction of both adrenal glands. About 70% of reported cases of primary adrenal insufficiency result from destruction of the adrenal cortex, the outer layer of the adrenal glands, by the body's own immune system.
Nowadays, the major cause of Addison's disease is an auto-immune reaction in which the body's immune system erroneously makes antibodies against the cells of the adrenal cortex and slowly destroys them. That process takes months to years.
There are also several less common causes of Addison's disease:If, on the other hand, ACTH is deficient, there will not be enough cortisol produced, although aldosterone may remain adequate. This is secondary adrenal insufficiency, which is distinctly different, but similar to, Addison's disease, since both include a loss of cortisol secretion. Secondary adrenal insufficiency can result from prolonged or improper use of glucocorticoid hormones, such as prednisone, which are used to treat rheumatoid arthritis, asthma and other inflammatory illnesses. Less common causes include pituitary tumors and damage to the pituitary gland during surgery or radiation.
Signs & Symptoms
The slowly progressive loss of cortisol and aldosterone secretion usually produces a chronic, steadily worsening fatigue, a loss of appetite, and some weight loss. Blood pressure is low (hypotension) and falls further when a person is standing, producing lightheadedness, dizziness or fainting. Nausea, sometimes with vomiting, and diarrhea are common. The muscles are weak and often go into spasm. There are often emotional changes, particularly moodiness, irritability and depression. Because of salt loss, a craving for salty foods is common. Finally, the increase in ACTH due to the loss of cortisol will usually produce a blotchy darkening of the skin that may look like an inappropriate tan on a person who feels very sick. Skin discoloration is more noticeable on body parts exposed to the sun and on the forehead, knees and elbows; scars and skin folds and creases (such as on the palms).
Other symptoms include:- Inability to digest food (and therefore the weight loss)
- Blood sugar disorders, including hypoglycemia
- Inability to cope with stress
- Intolerance to heat or cold
Some of these symptoms may of course indicate conditions other than Addison's disease. If you experience any of the symptoms, talk with your doctor about whether Addison's disease or another condition may be the cause.
Because symptoms of Addison's disease progress slowly, they may go unrecognized until a physically stressful event, such as another illness (e.g. flu), surgery or an accident, causes them to become much worse suddenly. Such an acute episode of the disease is called an Addisonian crisis. In about 25% of patients with Addison's disease, symptoms first become apparent during this type of crisis. An Addisonian crisis is considered a medical emergency because, left untreated, it can be fatal. Symptoms of an Addisonian crisis include the following: - Sudden penetrating pain in the lower back, abdomen or legs
- Severe vomiting and diarrhea, followed by dehydration
- Low blood pressure
- Loss of consciousness
Diagnosis & Tests
An endocrinologist should be consulted as they are specialists in hormonal diseases, including Addison's disease. Because of the rarity of the disease, an endocrinologist will have more training and experience in properly diagnosing and treating Addison's disease than most physicians.
A medical history of the symptoms mentioned above, especially hyperpigmentation of the skin or gums, is often enough to raise a strong suspicion, prompting the appropriate tests. Quite often, however, the first clue is from the abnormal results of routine tests done in a hospital or doctor's office. These may include an elevated blood level of potassium, a low blood level of sodium, a shift in the ratio of certain white blood cells, or surprising changes on an EKG or chest X-ray that are caused by high potassium or low blood volume.
Other causes for these changes, particularly from medications, must be considered first. A definitive diagnosis of Addison's disease requires that definitive tests be carried out. These tests measure the amount of cortisol and aldosterone in the blood and urine, and document a lack of the normal increase in the levels of these two hormones after administration of ACTH given by injection. An elevated blood level of ACTH should also be found. If the patient is very sick and Addison's disease is suspected, treatment can be initiated while the diagnostic tests are being done. Once the diagnosis of Addison's disease is established, an effort should be made to find the cause by checking for tuberculosis and other infections through skin tests and X-rays.
Treatment & Prevention
Since all of the manifestations of Addison's disease are caused by the lack of cortisol and aldosterone, the treatment is to replace these with similar steroids. Cortisol is usually replaced orally by cortisone acetate or hydrocortisone tablets divided into morning and afternoon doses. Aldosterone is replaced by an aldosterone-like synthetic steroid, fludrocortisone (Florinef) tablets given once daily. The doses of each of these medications are adjusted for the individual's size and any co-existing medical conditions. In emergencies or during surgery, hydrocortisone must be given intravenously.
Patients with Addison's disease should be taught to treat minor illnesses with extra salt and fluids. This is especially important if fever, vomiting or diarrhea is present. Persistence of these signs requires immediate treatment in an emergency room with intravenous saline (salt water) and hydrocortisone. Since Addison's disease is a chronic condition, daily replacement medication can never be stopped.
Proper maintenance treatment requires regular visits to a physician for examinations, laboratory tests, and discussions about symptoms. Certain blood tests, including sodium, potassium, blood counts and plasma renin are very useful in monitoring the response to adjustments in dosage. There is no single blood or urine test that is sufficient by itself.
Prognosis; Seek medical attention if
As long as the proper dose of replacement medication is taken every day, an Addisonian can have a normal crisis-free life. There are no specific physical or occupational restrictions. Routine care includes regular physician visits, avoidance of dehydration, and the use of extra medication during illness. Pregnancy is possible, but will require extra monitoring of the replacement medication. Every Addisonian should wear an identification bracelet or necklace stating that he or she has the disease, to insure proper emergency treatment. An identification card outlining treatment is also suggested. Today, people with Addison's disease should have a normal life expectancy.
See a doctor if you notice darkening of your skin or gums, along with other symptoms of Addison's disease.
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Conditions that suggest Addison's Disease:
Risk factors for Addison's Disease: | | | | | Autoimmune |  Autoimmune Tendency |
Infections |
Tuberculosis | When Dr. Thomas Addison first described this disease in London in 1855, the most common cause was tuberculosis. This remained the leading cause until the middle of the twentieth century when antibiotics progressively reduced TB's incidence. TB is nevertheless still a possible cause. |
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KEY | | Weak or unproven link |  | | Proven definite or direct link |
GLOSSARY
Abdomen (Abdominal)
That part of the body between the chest and the hips that contains the stomach, intestines, liver, bladder, pancreas and other organs.
Acute
An illness or symptom of sudden onset, which generally has a short duration.
Addison's Disease
A condition characterized by the chronic destruction of the adrenal cortex, which leads to an increased loss of sodium and water in the urine, muscle weakness and low blood pressure. The bronze color of the skin is due to the increased production of the skin pigment, melanin.
Adrenal (Adrenal Gland, Adrenal Glands, Adrenals)
The adrenal glands sit on top of each kidney and consist of an outer cortex and an inner medulla. Of the 50 or so hormones the adrenals make, only cortisone and adrenaline are recognized by most people. Some of these hormones must be produced to preserve life, while others help resist stress. Other hormones from the adrenals control normal energy output (along with the thyroid) and govern the breakdown of stored energy into quick energy sources. The medulla produces epinephrine and norepinephrine, which are specifically designed to help the body deal with stressful situations. The adrenals control the body's potassium/sodium balance, which is vital for energy production. They also produce sex hormones in minute amounts, which is important for later years when the gonads drop or cease their production.
Adrenal Cortex
The outer covering of the two adrenal glands that lie atop each kidney. Embryonically derived from gonad tissue, they make steroid hormones that control electrolytes, the management of fuels, the rate of anabolism, mineral metabolism, glucose metabolism, the general response to stress, and maintenance of nonspecific resistance.
Adrenal Insufficiency (Adrenal Exhaustion, Low Adrenal Function)
A condition in which the adrenal gland is compromised in its production of epinephrine, norepinephrine, cortisol, corticosterone or aldosterone. Symptoms include primarily fatigue, weakness, decreased appetite with ensuing weight loss, as well as nausea, vomiting, abdominal pain, diarrhea or constipation, or increased pigmentation of the skin. Cortical insufficiency (low or no corticosteroids) produces a more serious condition called Addison’s Disease, characterized by extreme weakness, low blood pressure, pigmentation of the skin, shock or even death.
Adrenal Medulla
The inner part of the adrenals, derived embryonically from spinal nerve precursors, they secrete epinephrine, norepinephrine and dopamine; used locally as neurotransmitters, sensitive receptors can be mobilized totally by the adrenal medullas.
Adrenaline (Epinephrin, Epinephrine)
A hormone secreted by the adrenal medulla that is released into the bloodstream in response to physical or mental stress, as from fear or injury. It initiates many bodily responses, including the stimulation of heart action and an increase in blood pressure, metabolic rate, and blood glucose concentration.
AIDS
Acquired Immune Deficiency Syndrome. An immune system deficiency disorder that suddenly alters the body's ability to defend itself. The AIDS virus invades the T4 helper/inducer lymphocytes and multiplies, causing a breakdown in the body's immune system, eventually leading to overwhelming infection and/or cancer, with ultimate death.
Aldosterone
A steroid hormone secreted by the adrenal cortex that helps the kidneys regulate the amount of salt and water in your body and, thus, regulate your blood pressure. When aldosterone levels drop too low, your kidneys cannot keep your salt and water levels in balance, and your blood volume and blood pressure drop.
Antibody (Antibodies)
A type of serum protein (globulin) synthesized by white blood cells of the lymphoid type in response to an antigenic (foreign substance) stimulus. Antibodies are complex substances formed to neutralize or destroy these antigens in the blood. Antibody activity normally fights infection but can be damaging in allergies and a group of diseases that are called autoimmune diseases.
Asthma (Asthmatic)
A lung disorder marked by attacks of breathing difficulty, wheezing, coughing, and thick mucus coming from the lungs. The episodes may be triggered by breathing foreign substances (allergens) or pollutants, infection, vigorous exercise, or emotional stress. Extrinsic Asthma is triggered by pollen, chemicals or some other external agent; Intrinsic Asthma is triggered by boggy membranes, congested tissues, or other native causes… even adrenalin stress or exertion.
Cancer
Refers to the various types of malignant neoplasms that contain cells growing out of control and invading adjacent tissues, which may metastasize to distant tissues.
Chronic (Chronicity)
Usually referring to chronic illness: Illness extending over a long period of time.
Cobalamin (B12, B-12, Cobalamine, Vitamin B12)
Essential for normal growth and functioning of all body cells, especially those of bone marrow (red blood cell formation), gastrointestinal tract and nervous system, it prevents pernicious anemia and plays a crucial part in the reproduction of every cell of the body i.e. synthesis of genetic material (DNA).
Corticosteroid (Corticosteroids)
Steroid hormone produced by the adrenal cortex.
Cortisol
A hormone. Its most important function is to help the body respond to stress. It also helps regulate your body's use of protein, carbohydrates and fat; it helps maintain blood pressure and cardiovascular function; it stems inflammation.
Crave (Craving, Cravings)
To have a strong desire for; to feel the need for.
Cytomegalovirus (CMV)
A member of the herpes virus family which may induce the immune-deficient state or cause active illness, such as pneumonia, in a patient already immune-deficient due to chronic illness, such as cancer or organ transplantation therapy.
Diabetes Mellitus (Diabetes, Diabetic, Diabetics)
A disease with increased blood glucose levels due to lack or ineffectiveness of insulin. Diabetes is found in two forms; insulin-dependent diabetes (juvenile-onset) and non-insulin-dependent (adult-onset). Symptoms include increased thirst; increased urination; weight loss in spite of increased appetite; fatigue; nausea; vomiting; frequent infections including bladder, vaginal, and skin; blurred vision; impotence in men; bad breath; cessation of menses; diminished skin fullness. Other symptoms include bleeding gums; ear noise/buzzing; diarrhea; depression; confusion.
Diarrhea
Excessive discharge of contents of bowel.
Electrocardiogram (ECG, EKG)
A test that shows a tracing of the electrical conduction of the heart.
Endocrine Glands
A set of glands including the pituitary, hypothalamus, pineal, thyroid, parathyroid, thymus and adrenal glands, as well as the pancreas, ovaries and testes.
Gland (Glands)
The glandular system is one of the most important and complicated systems of the body. Gland tissue can be either an organ or general tissue that secretes chemicals and there are two types of gland: exocrine and endocrine. Those glands which secrete chemicals through tubules or ducts are called exocrine and include sweat, tear and salivary glands. Ductless glands - part of the endocrine system - secrete special chemicals (hormones) directly into the blood.
Glucocorticoid
Any steroid-like compound capable of significantly influencing intermediary metabolism, such as promotion of deposition of glycogen in the liver, and of exerting a useful antiinflammatory effect.
Gonads
Both male and female reproductive glands are also called gonads: female ovaries and male testes both produce the same hormones, just in different amounts. These include estrogen, progesterone, and testosterone. These hormones amplify sexual characteristics that are dormant before puberty. The danger of the wrong proportions of hormones from these glands is that their function, rather than helping to bring about new life, may encourage improper cellular changes that may become life-threatening. Nutritional needs for the gonads vary somewhat, but all require plenty of essential fatty acids, vitamins C, E and B-complex, the minerals calcium, magnesium, selenium, iron and zinc.
Hemorrhage (Hemorrhaging)
Profuse blood flow.
Hormones (Hormone)
Chemical substances secreted by a variety of body organs that are carried by the bloodstream and usually influence cells some distance from the source of production. Hormones signal certain enzymes to perform their functions and, in this way, regulate such body functions as blood sugar levels, insulin levels, the menstrual cycle, and growth. These can be prescription, over-the-counter, synthetic or natural agents. Examples include adrenal hormones such as corticosteroids and aldosterone; glucagon, growth hormone, insulin, testosterone, estrogens, progestins, progesterone, DHEA, melatonin, and thyroid hormones such as thyroxine and calcitonin.
Hypoglycemia
A condition characterized by an abnormally low blood glucose level. Severe hypoglycemia is rare and dangerous. It can be caused by medications such as insulin (diabetics are prone to hypoglycemia), severe physical exhaustion, and some illnesses.
Hypotension
Low blood pressure.
Hypothyroidism (Hypothyroid)
Diminished production of thyroid hormone, leading to low metabolic rate, tendency to gain weight, and sleepiness.
Immune System (Immune Response, Immunity)
A complex that protects the body from disease organisms and other foreign bodies. The system includes the humoral immune response and the cell-mediated response. The immune system also protects the body from invasion by making local barriers and inflammation. The process may involve acquired immunity (the ability to learn and remember a specific infectious agent), or innate immunity (the genetically programmed system of responses that attack, digest, remove, and initiate inflammation and tissue healing).
Kidneys (Kidney, Renal)
Bean-shaped organs, each about the size of a fist. They are located near the middle of the back, just below the rib cage. The kidneys are sophisticated reprocessing machines, each day handling about 50 gallons of blood to sift out about half a gallon of waste products and extra water. The waste and extra water become urine, which flows to the bladder through tubes called ureters. The actual filtering occurs in tiny units inside the kidneys called nephrons. Every kidney has about a million nephrons. In a nephron, a glomerulus -- which is a tiny blood vessel, or capillary -- intertwines with a tiny urine-collecting tube called a tubule. A complicated chemical exchange takes place, as waste materials and water leave your blood and enter your urinary system. The kidneys recycle chemicals such as sodium, phosphorus, and potassium and thus regulate their levels. Renal: Pertaining to the kidneys.
Liver (Hepatic)
The largest and one of the most complex organs of the body, the liver is responsible for much of the metabolism of fats, proteins and carbohydrates. It is the site of much of the body's detoxification. It is connected very closely with digestion and the regulation of blood sugar, among many other functions. Found behind the ribs on the right side of the abdomen, it has many important functions such as removing harmful material from the blood, making enzymes and bile that help digest food, and converting food into substances needed for life and growth. Hepatic: Pertaining to the liver.
Nausea
Symptoms resulting from an inclination to vomit.
Parathyroid (Parathyroid Gland, Parathyroid Glands, Parathyroids)
The four parathyroid glands are embedded near the thyroid, with two attached to each thyroid lobe, on opposites sides of the throat. Hormones from the parathyroid glands help control calcium and phosphorous levels in the body. These glands oversee the breaking down of bone cells to release more of these minerals into the blood; a hormone from the thyroid has the opposite effect, and builds up the bones. Control of this system depends on blood levels of calcium ions: low levels "turn on" hormone production to break down bone tissue, so that blood levels remain stable. It is important to absorb enough dietary calcium to avoid this breakdown of bone tissue.
Pernicious Anemia
Anemia caused by a vitamin B12 deficiency.
Pituitary (Pituitary Gland)
The pituitary gland is a small (half-inch), bean-shaped organ that hangs down from the lower center of the brain on a stalk attached to another gland, the hypothalamus. Weighing less than one gram, the pituitary gland is often called the "master gland" since it controls the secretion of hormones by other endocrine glands. It regulates many body activities, and is partitioned into front and back lobes. The front lobe is stimulated by the hypothalamus, and produces any one of six different hormones that in turn stimulate the thyroid, adrenal and reproductive glands, and also breast milk production.
Potassium
A mineral that serves as an electrolyte and is involved in the balance of fluid within the body. Our bodies contain more than twice as much potassium as sodium (typically 9oz versus 4oz). About 98% of total body potassium is inside our cells. Potassium is the principal cation (positive ion) of the fluid within cells and is important in controlling the activity of the heart, muscles, nervous system and just about every cell in the body. Potassium regulates the water balance and acid-base balance in the blood and tissues. Evidence is showing that potassium is also involved in bone calcification. Potassium is a cofactor in many reactions, especially those involving energy production and muscle building.
Rheumatoid Arthritis
A long-term, destructive connective tissue disease that results from the body rejecting its own tissue cells (autoimmune reaction).
Sodium
An essential mineral that our bodies regulate and conserve. Excess sodium retention increases the fluid volume (edema) and low sodium leads to less fluid and relative dehydration. The adult body averages a total content of over 100 grams of sodium, of which a surprising one-third is in bone. A small amount of sodium does get into cell interiors, but this represents only about ten percent of the body content. The remaining 57 percent or so of the body sodium content is in the fluid immediately surrounding the cells, where it is the major cation (positive ion). The role of sodium in the extracellular fluid is maintaining osmotic equilibrium (the proper difference in ions dissolved in the fluids inside and outside the cell) and extracellular fluid volume. Sodium is also involved in nerve impulse transmission, muscle tone and nutrient transport. All of these functions are interrelated with potassium.
Spasm
Involuntary contraction of one or more muscle groups.
Steroid (Steroids)
Any of a large number of hormonal substances with a similar basic chemical structure containing a 17-carbon 14-ring system and including the sterols and various hormones and glycosides.
Syndrome
A medical condition characterized by a collection of related symptoms (what the patient feels) and signs (what a doctor can observe or measure).
Thyroid (Thyroid Gland)
The thyroid gland is an organ with many veins, anchored around the front of the throat near the voice box. It is essential to normal body growth in infancy and childhood. It absorbs iodine from the diet and releases thyroid hormones - iodine-containing compounds that help govern the rate of the body's metabolism (its total life processes), affecting body temperature, and regulating protein, fat and carbohydrate catabolism in all cells. They keep up growth hormone release, skeletal maturation, and heart rate, force, and output. They promote central nervous system growth, stimulate the making of many enzymes, and are necessary for muscle tone and vigor. To a high degree, metabolism is regulated by the hormone thyroxine, which can be made by the thyroid if enough organic iodine is available. An enlarged thyroid gland that is not cancer is sometimes called goitre.
Tuberculosis
Also known as TB, Consumption or "The White Plague", tuberculosis is an infectious disease caused by a bacterium called Mycobacterium tuberculosis, usually affecting the lungs but possibly also the brain, kidneys and bones. Patients may at first be symptom-free or experience a flu-like illness. In the secondary stage, there might be a slight fever, night sweats, weight loss, fatigue and various other symptoms, depending on the part of the body affected. Tuberculosis of the lung is usually associated with a dry cough that eventually leads to a productive cough with blood-stained sputum. There might also be chest pain and shortness of breath.
Virus (Viri, Viruses)
Any of a vast group of minute structures composed of a protein coat and a core of DNA and/or RNA that reproduces in the cells of the infected host. Capable of infecting all animals and plants, causing devastating disease in immunocompromised individuals. Viruses are not affected by antibiotics, and are completely dependent upon the cells of the infected host for the ability to reproduce.
White Blood Cell (WBC, White Blood Cells)
A blood cell that does not contain hemoglobin: a blood corpuscle responsible for maintaining the body's immune surveillance system against invasion by foreign substances such as viruses or bacteria. White cells become specifically programmed against foreign invaders and work to inactivate and rid the body of a foreign substance. White blood cells are composed primarily of neutrophils, monocytes and lymphocytes. Lymphocytes are either T-cells or B-cells. T-cells (CD3 cells) are divided into T-helper (CD4 cells) and T-suppressor/cytotoxic (CD8 cells) cells.
X-rays (X-ray)
High-energy radiation used to take pictures of areas inside the body.
Last updated: Apr 13, 2008
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