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Addison's Disease: Acute Adrenal Failure (Addisonian Crisis)

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Addison's Disease

Definition
Addison's disease is a disorder that results when the body produces insufficient
amounts of certain hormones produced by the adrenal glands. In Addison's disease,
the adrenal glands produce too little cortisol, and often insufficient levels of
aldosterone as well.

Also called adrenal insufficiency or hypocortisolism, Addison's disease can occur at


any age, but is most common in people ages 30 to 50. Addison's disease can be life-
threatening.

Treatment for Addison's disease involves the taking hormones to replace the
insufficient amounts being made by the adrenal glands, in order to mimic the
beneficial effects those naturally made hormones would normally produce.

Symptoms
Addison's disease symptoms usually develop slowly, often over several months, and
may include:

 Muscle weakness and fatigue


 Weight loss and decreased appetite
 Darkening of your skin (hyperpigmentation)
 Low blood pressure, even fainting
 Salt craving
 Low blood sugar (hypoglycemia)
 Nausea, diarrhea or vomiting
 Muscle or joint pains
 Irritability
 Depression

Acute adrenal failure (addisonian crisis)


Sometimes, however, the signs and symptoms of Addison's disease may appear
suddenly. In acute adrenal failure (addisonian crisis), the signs and symptoms may
also include:

 Pain in your lower back, abdomen or legs


 Severe vomiting and diarrhea, leading to dehydration
 Low blood pressure
 Loss of consciousness
 High potassium (hyperkalemia)
Causes

Adrenal glands

Your adrenal glands are located just above each of your two kidneys. These glands
are part of your endocrine system, and they produce hormones that give instructions
to virtually every organ and tissue in your body.

Your adrenal glands are composed of two sections. The interior (medulla) produces
adrenaline-like hormones. The outer layer (cortex) produces a group of hormones
called corticosteroids, which include glucocorticoids, mineralocorticoids and male sex
hormones (androgens).

Some of the hormones the cortex produces are essential for life — the glucocorticoids
and the mineralocorticoids.

 Glucocorticoids. These hormones, which include cortisol, influence your body's ability to
convert food fuels into energy, play a role in your 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 and water to keep your blood pressure normal.

Primary adrenal insufficiency


Addison's disease occurs when the cortex is damaged and doesn't produce its
hormones in adequate quantities. Doctors refer to the condition involving damage to
the adrenal glands as primary adrenal insufficiency.

The failure of your adrenal glands to produce adrenocortical hormones is most


commonly the result of the body attacking itself (autoimmune disease). For unknown
reasons, your immune system views the adrenal cortex as foreign, something to attack
and destroy.
Other causes of adrenal gland failure may include:

 Tuberculosis
 Other infections of the adrenal glands
 Spread of cancer to the adrenal glands
 Bleeding into the adrenal glands

Secondary adrenal insufficiency


Adrenal insufficiency can also occur if your pituitary gland is diseased. The pituitary
gland makes a hormone called adrenocorticotropic hormone (ACTH), which
stimulates the adrenal cortex to produce its hormones. Inadequate production of
ACTH can lead to insufficient production of hormones normally produced by your
adrenal glands, even though your adrenal glands aren't damaged. Doctors call this
condition secondary adrenal insufficiency.

Another more common possible cause of secondary adrenal insufficiency occurs


when people who take corticosteroids for treatment of chronic conditions, such as
asthma or arthritis, abruptly stop taking the corticosteroids.

Addisonian crisis
If you have untreated Addison's disease, an addisonian crisis may be provoked by
physical stress, such as an injury, infection or illness.

When to seek medical advice


See your doctor if you have signs and symptoms that commonly occur in people with
Addison's disease. Most people with this condition experience darkening areas of skin
(hyperpigmentation), severe fatigue, unintentional weight loss, and gastrointestinal
problems, such as nausea, vomiting and abdominal pain. Dizziness or fainting, salt
cravings, and muscle or joint pains also are common.

Your doctor can determine whether Addison's disease or some other medical
condition may be causing these problems.

Tests and diagnosis


Your doctor will talk to you first about your medical history and your signs and
symptoms. If your doctor thinks that you may have Addison's disease, you may
undergo some of the following tests:

 Blood test. Measuring your blood levels of sodium, potassium, cortisol and
ACTH gives your doctor an initial indication of whether adrenal insufficiency
may be causing your signs and symptoms. A blood test can also measure
antibodies associated with autoimmune Addison's disease.
 ACTH stimulation test. This test involves measuring the level of cortisol in
your blood before and after an injection of synthetic ACTH. ACTH signals
your adrenal glands to produce cortisol. If your adrenal glands are damaged,
the ACTH stimulation test shows that your output of cortisol in response to
synthetic ACTH is blunted or nonexistent.
 Insulin-induced hypoglycemia test. Occasionally, doctors suggest this test if
pituitary disease is a possible cause of adrenal insufficiency (secondary
adrenal insufficiency). The test involves checking your blood sugar (blood
glucose) and cortisol levels at various intervals after an injection of insulin. In
healthy people, glucose levels fall and cortisol levels increase.
 Imaging tests. Your doctor may have you undergo a computerized
tomography (CT) scan of your abdomen to check the size of your adrenal
glands and look for other abnormalities that may give insight to the cause of
the adrenal insufficiency. Your doctor may also suggest a CT scan or MRI
scan of your pituitary gland if testing indicates you have secondary adrenal
insufficiency.

Treatments and drugs


If you receive an early diagnosis of Addison's disease, treatment may involve taking
prescription corticosteroids. Because your body isn't producing sufficient steroid
hormones, your doctor may have you take one or more hormones to replace the
deficiency. Cortisol is replaced using hydrocortisone (Cortef), prednisone or cortisone
acetate. Fludrocortisone (Florinef) replaces aldosterone, which controls your body's
sodium and potassium needs and keeps your blood pressure normal.

You take these hormones orally in daily doses that mimic the amount your body
normally would make, thereby minimizing side effects. If you're facing a stressful
situation, such as an operation, an infection or a minor illness, your doctor will
suggest a temporary increase in your dosage. If you're ill with vomiting and can't
retain oral medications, you may need corticosteroid injections.

In addition, your doctor may recommend treating androgen deficiency with an


androgen replacement called dehydroepiandrosterone. Some studies indicate that, for
women with Addison's disease, androgen replacement therapy may improve overall
sense of well-being, libido and sexual satisfaction.

Addisonian crisis
An addisonian crisis is a life-threatening situation that results in low blood pressure,
low blood levels of sugar and high blood levels of potassium. This situation requires
immediate medical care. Treatment typically includes intravenous injections of:

 Hydrocortisone
 Saline solution
 Sugar (dextrose)

Coping and support


These steps may help you cope better with a medical emergency if you have
Addison's disease:

 Carry a medical alert card and bracelet at all times. In the event you're
incapacitated, emergency medical personnel know what kind of care you need.
 Keep extra medication handy. Because missing even one day of therapy may
be dangerous, it's a good idea to keep a small supply of medication at work, at
a vacation home and in your travel bag, in the event you forget to take your
pills. Also, have your doctor prescribe a needle, syringe and injectable form of
corticosteroids to have with you in case of an emergency.
 Stay in contact with your doctor. Keep an ongoing relationship with your
doctor to make sure that the doses or replacement hormones are adequate but
not excessive. If you're having persistent problems with your medications, you
may need adjustments in the doses or timing of the medications.

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