Article: Addison's disease

Addison's disease (also known as chronic adrenal insufficiency, or hypocortisolism) is a rare endocrine disorder which results in the body not producing sufficient amounts of certain adrenal hormones. The condition was first described by British physician Thomas Addison in his 1855 publication: On the Constitutional and Local Effects of Disease of the Suprarenal Capsules. Those who have Addison's are often referred to in research and informational articles as "Addisonians."

Characteristics

Addison's disease refers specifically to primary adrenal insufficiency, in which the adrenal glands themselves malfunction; secondary adrenal insufficiency occurs when the anterior pituitary gland does not produce enough adrenocorticotropic hormone (ACTH) to adequately stimulate the adrenal glands.

Cause

Addison's Disease is caused by the failure of the adrenal glands, seated above the kidneys, to produce enough of the hormone cortisol and, in some cases, the hormone aldosterone.

Signs and symptoms

Addison's disease usually develops slowly (over several months), and symptoms may not present or be noticed until some stressful illness or situation occurs. Common symptoms are:

  • Chronic fatigue that gradually worsens
  • Muscle weakness
  • Weight loss and Loss of appetite
  • Nausea, diarrhea, or vomiting
  • Low blood pressure that falls further when standing (orthostatic hypotension)
  • Areas of hyperpigmentation (darkened skin), known as melasma suprarenale.
  • Irritability
  • Depression
  • Craving for salt and salty foods
  • Hypoglycemia, low blood sugar (worse in children)
  • For women, menstrual periods that become irregular or cease
  • Tetany (particularly after drinking milk) due to phosphate excess
  • Numbness of the extremities, sometimes with paralysis, due to potassium excess
  • Increased number of eosinophils
  • Polyuria

Addisonian crisis

In some cases, Addison's symptoms may present rapidly. This "acute adrenal failure" is known as an Addisonian crisis and is a severe medical emergency. An illness or accident can aggravate the adrenal problems causing the Addisonian crisis (most common in untreated sufferers), although the most common cause (for those already diagnosed) is abrupt discontinuation of corticosteroid therapy without tapering the dose.

Untreated, an Addisonian crisis can be fatal. It is a medical emergency.

Symptoms that may occur include:

  • Sudden penetrating pain in the legs, lower back or abdomen
  • Severe vomiting and diarrhea, resulting in dehydration
  • Low blood pressure
  • Loss of consciousness
  • Hypoglycemia
  • Brown coating on tongue and teeth due to iron loss (hemolysis)

Frequency

The frequency rate of Addison's disease in the human population is usually estimated at roughly 1 in 100,000. [1] Some research and information sites put the number closer to 40-60 cases per 1 million population. (1/25,000-1/16,600)[2] (Determining accurate numbers for Addison's is problematic at best and some incidence figures are thought to be underestimates. [3]) Addison's can afflict persons of any age, gender, or ethnicity, but typically presents in adults between 30 and 50 years of age. Women are slightly more likely to develop Addison's according to some studies. Research has shown no significant predispositions based on ethnicity. [4]

Heredity

Addison's Disease is generally not believed to be passed on in humans through genetics. Some research has shown that canine Addison's may have a genetic component. Further, some of the initial causes of Addison's may have genetic components that contribute to, but cannot be considered the sole cause of, Addison's Disease.

Medical procedures

Tha Mayo Clinic urges one to seek medical advice for potential Addison's Disease "If you have severe fatigue, have unintentionally lost weight, feel progressively weaker, experience abdominal pain, have fainting spells and your skin has become darker, see your doctor to determine whether Addison's disease or some other medical condition may be the cause." [5]

Diagnosis

In suspected cases of Addison's disease, one needs to demonstrate that adrenal hormone levels are low after appropriate stimulation with synthetic pituitary hormone.

Once demonstrated, the cause of adrenal failure needs to be elucidated. The most common cause is autoimmune, and can be tested for with an assay for 21-hydroxylase antibodies. If there are no antibodies present, infectious or genetic causes should be sought. This may include imaging of the adrenal glands, tests for tuberculosis or HIV infection, and searching for metastatic cancer.

Primary adrenal insufficiancy

Also known as: Primary Addison's Disease. Primary Addison's Disease is caused by damaged adrenal glands where the damage causes the insufficient production of the above-mentioned hormones. Most often the damage is caused by autoimmune disease, where the body creates antibodies that attack the glands (as if it were a disease) in the same way the immune system fights infection. Other causes of failure of the adrenal glands may include the following:

  • Tuberculosis
  • Infections of the adrenal glands
  • Spread of cancer to the adrenal glands
  • Bleeding into the adrenal glands

Secondary adrenal insufficiancy

Also known as: Secondary Addison's Disease. Often caused by a diseased pituitary gland or when a person on corticosteroid medications for chronic conditions (e.g. arthritis, asthma) abruptly cease taking the medicines. Secondary Addison's Disease is characterized by inadequate production of the pituitary hormone called adrenocorticotropic hormone, ACTH. ACTH triggers production of adrenal hormones and a shortage of ACTH can cause a shortage of the adrenal hormones, even though the adrenal glands themselves are not damaged.

Etiology

Eighty to ninety percent of cases of Addison's disease are said to be due to autoantibodies directed against adrenal cells containing 21-hydroxylase, an enzyme involved in the production of cortisol and aldosterone.

The remainder of cases are due to tuberculosis, HIV, sarcoidosis, amyloidosis, hemochromatosis, metastatic cancer to the adrenal glands, adrenal haemorrhage, Waterhouse-Friderichsen syndrome (massive, usually bilateral, hemorrhage into the adrenal glands caused by fulminant meningococcemia,) and congenital adrenal hyperplasia.

Addison's disease can be an expression of an autoimmune polyendocrine syndrome when autoimmune reactions against other organs are also present. In APS type 1, 70% suffer from Addison's disease, while in type 2, 100% do (by definition).

Through these syndromes, Addison's is associated with hypothyroidism, diabetes mellitus (type 1), vitiligo, alopecia and celiac disease.

Treatment

Treatment for Addison's disease involves replacing the missing cortisol and, if necessary, fludrocortisone as replacement for the missing aldosterone. Caution must be exercised when the person with Addison's disease has surgery or becomes pregnant. Treatment for an acute attack - an Addisonian Crisis - usually involves intravenous (into blood veins) injections of:

  • Hydrocortisone
  • Saline solution (basically a salt water, same clear IV bag as used to treat dehydration)
  • Dextrose (a type of sugar)

Prognosis

While treatment solutions for Addison's Disease are far from precise, overall long-term prognosis is typically good. Because of individual physiological differences, each person with Addison's must work closely with their physician to adjust their medication dosage and schedule to find the most effective routine. Once this is accomplished (and occasional adjustments must be made from time to time, especially during periods of travel, stress, or other medical conditions), symptomology is usually greatly reduced or occasionally eliminated so long as the person continues their dosage schedule accurately.

Life activities

Those with Addisonian's are strongly urged to adhere to the following lifestyle necessities (in a medical emergency, these may save the person's life):

  • Wear a medical alert bracelet or necklace and carry a medical information card at all times. This card should also instruct emergency medical personnel to inject 100 mg of cortisol should the bearer be found injured or unable to answer questions, as well as provide the name and phone number of the person's physician and nearest relative or other emergency contact. [6]
  • Always have extra medication handy, including a Crisis Kit.
  • Stay in contact with your doctor. When travelling, insure a close friend or relative knows where you are and where you are heading (also, see the Travelling section below).

Daily challenges

For the person with Addison's Disease, simple life tasks may be quite difficult, if not impossible. With the proper treatment, though, most people can carry out a highly functional life with only infrequent major interruptions. Generally, there are no specific physical or occupational restrictions for an Addisonian. Additionally, those with Addisonian's Disease have a normal lifespan expectancy. [7]

Athletics

Addisonians are, by-and-large, capable of participating in a wide variety of sports. Some may find that their endurance is less then prior to the onset of Addison's Disease, but others may notice very little difference at all. Like treatment regimens, activity level and residual effects on life activities are different from Addisonian to Addisonian.

Travel

When an Addison's Disease sufferer travels, it is imperative that they create or maintain an emergency "Crisis Kit." If traveling with a companion, it is usually recommended that the companion be both able and willing to give the Addisonian a hyperdermic medication shot should it become necessary. With proper planning - including maintaining prescribed dosing schedules - travelling for those afflicted with Addisonians poses only minimal risks.

Relationships

People with Addison's Disease typically are just as capable as others in forming all manner of relationships, from friendships to business contacts and intimate/romantic relationships. Different (and additional) from a regular relationship, though, those in relationships with an Addisonian, expecially intimate/romantic relationships and close friends, is the potential opportunity for the Addisonian to explain what the condition is and basic emergency reactions in the case of an Addisonian Crisis in the presence of one of these close relationships.

High-risk events

Surgery

Surgeries may require significant adjustments to medication regimens prior to, during, and following any surgical procedure. The best preparation for any surgery, regardless of how minor or routine it may normally be, is to speak to one's primary physician about the procedure and medication implications well in advance of the surgery.

Pregnancy

Many, many women with Addison's have given birth successfully and without complication, both through "natural" labor and through cesarian delivery. Both of these methods will require different preventative measures relating to Addison's medications and dosages. As is always the case, thorough communication with one's primary physician is the best course of action. Occasionally, oral intake of medications will cause debilitating nausea and vomiting, and thus the woman may be switched to injected medications until delivery. [8] Addison's treatment courses by the mother are generally considered safe for baby during pregnancy.

Canine Addison's Disease

The condition is rare in dogs, but it has been diagnosed in the Bearded Collie and Chihuahua breeds, and in rare cases in Standard Poodles. In dogs it is inherited via the female line - possibly due to autoimmune antibodies in the milk.

The first sign of Addison's Disease is that the dog urinates much longer than normally. Urine is very light and pressure while urinating is lower than normal. When the dog loses its appetite and changes its temperament, a blood test must be taken as soon as possible.

Addison's disease is treated with Prednisolone and Florinef. The dog must have its medicine twice a day after a meal. The amount of the medicine required depends on the weight of the dog. Even if the dog feels good, it is absolutely necessary to take blood samples every 3-4 months, to make sure the dog's condition is stable. An alternative to oral Florinef is a monthly injection called Percorten V. The active ingredient is DOCP (Desoxycorticosterone Pivalate).

One of the medicines makes the dog drink more and the other one makes it urinate more. It is absolutely vital to provide fresh drinking water for the canine sufferer.

The owner of the ill dog, should measure the dog's pulse while it is relaxed. In stressful situations it is necessary to know when the dog's heart is pumping too fast and needs relaxing. If the owner knows about an upcoming stressful situation (shows, travelling etc.), they may give a larger dose of medicine.

The stress level of the ill dog must be as low as possible (both positive and negative stress). For example agility trainings, shows, guests etc. must be as stress-free as possible.

Famous Addisonians

President John F. Kennedy is one of the more well-known of Addison's Disease sufferers. Jane Austen is thought to have been another, although the disease had not been described during her lifetime. Charles Dickens is another famous writer who may have been afflicted, according to Dr. Carl Abbott, a Canadian medical researcher. [citation needed]

For more information...

Support groups

  • Addison's Disease Research Today - monthly online journal summarizing recent primary literature on Addison's Disease
  • Addison's Disease Self Help Group (ADSHG) - UK support group
  • Australian Addison's Disease Association - Australian support and information group

References

  • Thomas Addison [1855]. On The Constitutional And Local Effects Of Disease Of The Supra-Renal Capsules (HTML reprint), London: Samuel Highley.
  • Edward Headlam Greenhow [1866]. Addison's Disease: Clinical Lectures On Addison's Disease And A Report On Diseases Of The Supra-Renal Capsules (HTML reprint), Rotherhithe: J. W. Roche.
  • National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) (June 2004). NIH Publication No. 04–3054: Addison's disease. Endocrine and Metabolic Diseases Information Service. National Institue for Health. Retrieved on 2006-06-07.
  • Neuroimmunology, The Medical School, Birmingham University - Abid R Karim, Birmingham UK

External links

  • Overview of Addison's Disease from the Mayo Clinic
  • National Adrenal Diseases Foundation
  • eMedicine/WebMD article on Addison's Disease
  • Addison's information from MedicineNet
  • "Addison's Disease: The Facts You Need to Know (MedHelp.org)
  • Addison's Disease info from SeekWellness.com
  • Information about Addison's Disease in canines (dogs)
  • Addison's Disease website listing at Google
  • Addison's Disease website listing at Yahoo

See also

  • Cushing's syndrome
  • Type 1 Diabetes
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