Addison’S Disease

Addison disease occurs when the adrenal glands areno longer able to produce certain hormones needed for life, such ascortisol and aldosterone.

What is going on in the body?

There are two adrenal glands in the body, one on topof each kidney. They produce two hormones: cortisol and aldosterone.

Cortisol

Cortisol is a type of hormone called aglucocorticoid. It affects almost every organ and tissue in the body.Experts believe cortisol may have hundreds of effects, but its main job is to helpthe body respond to stress. Other vital tasks include the following.

It helps maintain blood pressure, heart function, and blood vessel function.

It helps slow the immune system’s inflammation response.

It helps balance the effects of insulin in breaking down glucose for energy.

It helps control the metabolism of proteins, carbohydrates, and fats.

Cortisol is vital to health, so the body maintains a precise balance.As with many other hormones, the hypothalamus and pituitary gland are keyto this control.

Aldosterone

Aldosterone is a type of hormone called a mineralcorticoid.It helps with the following functions:

maintains blood pressure

maintains water and salt balance in the body by helping the kidneys hangon to sodium and get rid of potassium

What are the causes and risks of the disease?

Addison disease is a rare disorder that affects only about 1 inevery 100,000 people. It occurs in all age groups, and affects both men andwomen equally. Addison disease may be caused by either a disorder of theadrenal glands themselves, which is called primary adrenal insufficiency. Or,it may be caused by inadequate secretion of ACTH by the pituitary gland.In primary adrenal insufficiency, an autoimmune disorderthat makes the person’s own immune system attack and destroy the outer layerof the adrenal glands, called the cortex, is often at fault. When at least 90 percentof the cortex has been destroyed, adrenal insufficiency occurs. Experts believe causes forthis type may include:

an inherited syndrome called polyendocrine deficiency syndrome, type 1 or type 2

tuberculosis, called TB

chronic infections, mainly fungal infections

cancerthat has spread from somewhere else in the body

amyloidosis

surgical removal of the adrenal glands

Secondary adrenal insufficiency is due to a lack of ACTH, which causes the adrenalglands to produce less cortisol. It does not affect production of aldosterone. Causesof this type of insufficiency include:

surgical removal of the benign, known as noncancerous, tumors of thepituitary gland linked with Cushing disease.These tumors produce excess ACTH, and when they are removed, thebody is unable to adapt quickly

hypopituitarism,due to tumors, infections, loss of blood flow to the pituitary, radiation forpituitary tumors, or surgical removal of parts of the hypothalamus or thepituitary gland

What are the signs and symptoms of the disease?

In most cases, the symptoms come on gradually.Addison disease commonly causes these symptoms:

skin changes with areas of dark tanning, which is most visible on scars, skinfolds, mucous membranes, and pressure points such as elbows, knees, andknuckles

low blood pressure,which may cause dizzinessor fainting if aperson stands up too fast

nausea, vomiting,and diarrhea

chronic, worsening muscle weaknessand fatigue

loss of appetiteand weight loss

irritability anddepression

craving of salty foods due to salt imbalance in the body

low blood glucose levels, called hypoglycemia,which is more severe in children than in adults

irregular or absent menstrual periods in women

If symptoms progress too long without treatment, the person maygo into an addisonian crisis. Symptoms of this crisis include:

sudden, piercing pain in the abdomen, lower back, or legs

severe vomitingand diarrhea

dehydration

dangerously low blood pressure

loss of consciousness

Without treatment, addisonian crisis can be fatal.

How is the disease diagnosed?

In its early stages, Addison disease can be hard to diagnose.Diagnosis of Addison disease begins with a medical historyand physical exam. Dark tanning of the skin is will often lead the doctorto suspect Addison disease. Blood tests, such as theACTH stimulation test,can confirm the low cortisol level and its effects on the body. X-ray examsof the adrenal and pituitary glands may be helpful in identifying the causeof the disease.

What can be done to prevent the disease?

Usually, nothing can be done to prevent Addison disease.People with certain infections such as tuberculosiscan sometimes avoid this condition if the infection is treated early.

What are the long-term effects of the disease?

Shockand death can result if the condition is not treated. With treatment, thereare generally no long-term effects. In life-threatening situations and illnesses,a person may need increased doses of hormones given as pills orshots to help the body adapt.

What are the risks to others?

Addison disease poses no risks to others.

What are the treatments for the disease?

Treatment of Addison disease involves replacing the missingadrenal hormones. Initial therapy may include IV fluids, othermedicines to support blood pressure or treat infections, and IVhormones. Once a person is stable, hormone pills alone can be used.Patients who need to replace aldosterone may also be advised by their doctorsto increase their salt intake.

What are the side effects of the treatments?

Medicines to support blood pressure or treat infectionsmay cause allergic reactionsand stomach upset. If hormones are given in too low or too high an amount,uncomfortable symptoms can result. For example, if adrenal hormone levels aretoo high, people can have mood swings and body swelling.

What happens after treatment for the disease?

In most cases, lifelong hormone replacement is required becauseadrenal function does not usually return to healthy levels once it is lost. Peoplewith Addison disease should wear or carry identification, such as a MedicAlert bracelet, describing their condition and emergency treatment needed.When people with this disease travel, they should bring along an injectable formof cortisol that can be used in case of emergency. A plan for increasing cortisolmedicine dosing during periods of high stress or with mild respiratoryinfections should also be discussed with the doctor.

How is the disease monitored?

Blood tests can be used to check for salt balance andhormone levels. Immediate medical attention will be needed if the persondevelops a severe infection or severe vomiting and diarrhea. These conditionscan bring on addisonian crisis. Any new or worsening symptoms should bereported to the doctor as well.