Definition

Addison's disease is a rare disorder of the adrenal glands. In the case of Addision's, the 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 adrenal cortex, the outer layer of the adrenal gland. This damage may be caused by:

• The body's immune system attacking the gland (autoimmune disease), which 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 (low blood pressure))
• Surgical complication
• Congenital (present at birth) or genetic factors (enzyme defects, familial glucocorticoid insufficiency)
• Cytomegalovirus (CMV) 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
  • Hemachromatosis
  • Amyloidosis
  • Adrenoleukodystrophy
  • Adrenomyelodystrophy

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition.

Risk factors include:

• Having the following autoimmune diseases can put you at risk for an associated autoimmune-based Addison’s disease:
  • 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 ACTH (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
• X-rays —pictures of the abdomen to see if the adrenal glands have signs of calcium deposits
• CT scan of the abdomen—a type of x-ray that uses a computer to make pictures of structures inside the body, in this case the adrenal glands

Treatment

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

Medications may include:

• Cortisone acetate
• Hydrocortisone tablets
• Fludrocortisone acetate (Florinef)

Immediate treatment of adrenal crisis includes:

• Hydrocortisone by IV (into vein)
• Normal saline (salt water) by IV

Prevention

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