What Are the Stages of Addison’s Disease?

Addison’s disease is caused by damage to the outermost layers of your adrenal glands. This is where your body makes the hormones aldosterone and cortisol.

Cortisol helps your body respond to stress. It also has several other important body functions:

• Maintain blood pressure and heart function
• Regulate water
• Control blood sugar levels

Aldosterone affects your body’s balance of sodium and potassium. If you don’t have enough aldosterone, it lowers your kidney’s ability to filter salt and water, resulting in low blood pressure.

In about 75% of people with Addison’s disease, this damage to the adrenal glands is due to an autoimmune reaction. This is when your body’s immune system wrongly attacks healthy tissue.

Tuberculosis used to be a major cause of Addison’s disease. This infectious disease can damage your adrenal glands. But this is a less common cause in developed countries once tuberculosis treatment improved.

Other causes of Addison’s disease include:

• Bleeding in your adrenal glands
• Cancer cells in your adrenal glands
• Genetic disorders that affect how your adrenal glands function or develop
• Adrenal gland removal
• Some medications like antifungal drugs

There are five stages of progression in Addison's disease:

• Stage 0: Normal adrenal function. 
• Stage 1: You may have increased plasma renin with normal or low levels of aldosterone but no other abnormal results. 
• Stage 2: You're likely to have high renin levels and low aldosterone levels. Renin is an enzyme produced in your kidney. It helps regulate your blood pressure. With an ACTH stimulation test, your cortisol levels may have low or no response.
• Stage 3: Your ACTH levels increase, and you may have normal or low cortisol levels. ACTH or adrenocorticotropic hormone is made by your pituitary gland. It stimulates your adrenal gland to produce cortisol.
• Stage 4: There are very high levels of ACTH and low cortisol levels. You may also have symptoms of Addison’s disease.

If you have Addison’s disease, but it’s undiagnosed and untreated, an Addisonian crisis (adrenal crisis) can happen. This is usually triggered by events of high stress such as trauma, accident, severe infection, or surgery. An Addisonian crisis is a medical emergency. 

Most cases of Addisonian crisis are in people with Addison’s disease who experience a high-stress event or who haven’t been taking their replacement hormone drugs. In a study of 444 people with Addison’s disease, 42% reported at least one Addisonian crisis.

Symptoms of an Addisonian crisis include:

• Severe pain in your lower back, legs, or abdomen
• Low blood pressure
• Loss of consciousness
• Sudden loss of strength
• Severe diarrhea and vomiting 

If not treated, an Addisonian crisis can lead to severe complications like:

• Cardiac arrest (your heart suddenly stops beating)
• Irregular heartbeat
• Loss of consciousness because of low blood pressure. This can progress to a state of shock with reduced blood flow to organs like your brain, liver, and heart.
• Low blood sugar and coma

Addison’s disease symptoms progress slowly and tend to be nonspecific. So it’s often confused with other illnesses.

Early-stage Addison’s disease symptoms include:

• Fatigue
• Nausea 
• Weight loss
• Dizziness when you stand up after lying down or sitting

Other symptoms of Addison’s disease include:

• Dark patches of skin, usually near scars, skin creases like your knuckles, and on mucous membranes like your gums (hyperpigmentation)
• White patches on your body (vitiligo) 
• Abdominal pain
• Vomiting
• Diarrhea 
• Muscle weakness 
• Muscle spasms and pain
• Joint pain
• Poor appetite 
• Dehydration
• Low blood pressure (hypotension)
• Low blood sugar levels 
• Depression
• Irritability 
• Low sex drive
• Irregular menstrual periods

Treatment for Addison's disease is medication. You'll need to take hormone drugs to replace what your body can't produce. The drug fludrocortisone replaces aldosterone, and cortisol is replaced by hydrocortisone.

If you have an Addisonian crisis, you’ll need immediate medical help. You’ll be injected with a high dose of hydrocortisone. You may also be given fluids, electrolytes, and vasopressors to help maintain your blood pressure.

You can’t prevent Addison’s disease, but you can take some steps to avoid an adrenal crisis:

• If you have Addison’s disease, always wear a medical alert wristband or carry a card stating you have the disease.
• Educate yourself, your family, and caregivers of the symptoms of an Addisonian crisis. Get immediate medical help if you develop these symptoms.
• Carry an emergency vial of injectable hydrocortisone with you. Make sure that you and your family members are trained to administer this injection.