Definition

Addison's disease is a rare disorder of the adrenal glands. With Addison's, the adrenal glands do not produce enough of the hormones cortisol and aldosterone.

Adrenal Glands

Addison's occurs because of damage to the cortex.

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Causes

Addison's disease is the result of gradual damage to the outer layer of the adrenal gland. This damage may be caused by:

• The body's own immune system attacking the gland. Known as an autoimmune disease. This cause accounts for 85% of cases in developed countries.
• Tuberculosis —major cause in the Third World countries
• Bleeding within the adrenal glands—related to use of anticoagulant medications and shock [extremely low blood pressure]
• Surgical complication
• Condition that are present at birth or due to genetic factors (enzyme defects, familial glucocorticoid insufficiency)
• Cytomegalovirus (CMV) infection associated with AIDS
• Fungal infections, including:
  • Blastomycosis
  • Histoplasmosis
  • Coccidioidomycosis
• Cancer including metastases from:
  • Breast, lung, kidney, or colon cancer
  • Lymphoma
  • Kaposi's sarcoma
• Medications (such as ketoconazole or etomidate)
• Radiation treatment
• Chronic illness, including:
  • Sarcoidosis
  • Hemochromatosis
  • Amyloidosis
  • Adrenoleukodystrophy
  • Adrenomyelodystrophy

Risk Factors

Factors that increases your chance of getting Addison's disease include:

• Having the following autoimmune diseases:
  • Type I diabetes
  • Pernicious anemia
  • Hypoparathyroidism
  • Hypopituitarism
  • Hyperthyroidism (Grave’s disease) or hypothyroidism (Hashimoto’s disease)
  • Myasthenia gravis
• Stress
• Anticoagulant medications
• Abdominal injury
• Family members with autoimmune-caused Addison's disease
• Long-term steroid medication treatment, followed by:
  • Severe stress
  • Infection
  • Surgery
  • Trauma
• Previous surgery on adrenal glands

Symptoms

Symptoms may include:

• Extreme weakness, fatigue
• Weight loss
• Nausea or vomiting
• Chronic diarrhea
• Muscle weakness
• Darkening of freckles, nipples, scars, skin creases, gums, mouth, nail beds, and vaginal lining
• Emotional changes, especially depression
• Craving for salty foods
• Abdominal pain
• Anorexia
• Amenorrhea

A severe complication of Addison's disease is the Addisonian or adrenal crisis. Adrenal crisis is a life threatening disorder, its symptoms include:

• Severe abdominal, back, or leg pain
• Fainting
• Severe low blood pressure
• Severe dehydration
• Severe nausea, vomiting, and diarrhea
• Low blood sugar
• Generalized muscle weakness

Diagnosis

The doctor will ask about your symptoms and medical history. A physical exam will be done.

Tests may include:

• Blood and urine tests—to see if you have low levels of cortisol and aldosterone, high level of adrenocorticotropic hormone (ACTH is a hormone that stimulates the adrenal glands), and to measure levels of:
  • Sodium
  • Chloride
  • Calcium
  • Potassium
  • Bicarbonate
  • Blood urea nitrogen levels
  • Anti-adrenal antibody (rarely done)
• ACTH stimulation test —measures cortisol in the blood before and after an injection of ACTH

Your doctor may also need images of the abdomen and adrenal glands. This images may be taken with:

• X-rays
• Computed tomography (CT) scan

Treatment

Symptoms of Addison's disease can be controlled with medicines. These drugs replace the missing hormones. Medicine needs to be taken for the rest of your life. They may need to be increased during times of stress.

Immediate treatment of adrenal crisis includes:

• Self-injection of dexamethasone
• Hydrocortisone by IV
• Normal saline by IV

Prevention

There are no guidelines for preventing Addison's disease. If you think you are at risk, talk to your doctor.