Adrenal Insufficiency – its symptoms, diagnosis and treatment are discussed in this article. Please note that Addison’s disease is a type of adrenal insufficiency. In the end, an actual case study of Addison’s disease further highlights how experienced endocrinologists manage this disease in real life.
What is adrenal insufficiency?
Adrenal insufficiency occurs when there is a significant decrease in the production of hormone cortisol by the adrenal glands. What are adrenal glands? Two small triangular structures lying on top of the kidneys.
Types of Adrenal Insufficiency:
There are two types:
1. Primary Adrenal Insufficiency
Primary adrenal insufficiency is also known as Addison’s disease. In this disease, there is a near- total destruction of both of the adrenal glands. Therefore, these patients have a very low level of cortisol as well as aldosterone.
2. Secondary Adrenal Insufficiency
Secondary adrenal insufficiency develops when there is a decrease in the hormone ACTH (Adreno-Cortico-Tropic- Hormone) from the pituitary gland in the brain.
ACTH regulates the production of cortisol by the adrenal gland. Naturally, a decrease in ACTH results in a decrease in the production of cortisol by the adrenal glands.
In secondary adrenal insufficiency, aldosterone production is normal as it is primarily under control of another hormonal mechanism, known as Renin Angiotensin Aldosterone axis.
What is the Most Common Cause of Adrenal Insufficiency?
In the developed countries, the most common cause of primary adrenal insufficiency is an autoimmune disease process – autoimmune adrenalitis. Often there is evidence of some other autoimmune disease as well such as Hashimoto’s thyroiditis, Type 1 diabetes or pernicious anemia. In the third world countries, however, tuberculosis is a common cause of this disease.
The most common cause of secondary adrenal insufficiency is a prolonged use of steroids such as prednisone or dexamethasone. Physicians often use steroids to treat a variety of diseases such as rheumatoid arthritis, asthma, lupus etc. If used more than three weeks, steroids can cause secondary adrenal insufficiency.
Other rare causes of secondary adrenal insufficiency include a tumor in the pituitary gland. The tumor itself or its treatment by surgery or radiation causes secondary adrenal insufficiency. These patients also develop deficiencies of several other hormones, especially thyroid hormones, sex hormones and growth hormone.
Symptoms of Adrenal Insufficiency
Fatigue is the main symptom – and often the only symptom. In addition, some patients may have low blood pressure and shortness of breath. Other symptoms include nausea, abdominal pain and weight loss. Some patients may also develop darkening of their skin and a craving for salt.
What is Adrenal Crisis or Addisonian Crisis?
If a patient does not receive treatment in time, then more severe signs develop: severe low blood pressure, low sodium and high potassium levels in the blood, shock and even death. This condition is also called Adrenal crisis or Addisonian crisis.
Diagnosis of Adrenal Insufficiency – Addison’s disease
Aa special test called Cortrosyn Stimulation test is the best way to diagnose Addison’s disease.
Cortrosyn Stimulation Test
In this test, a healthcare professional draws blood sample for a baseline cortisol level. Then, he/she injects Cortrosyn – synthetic ACTH – into the patient. Subsequently, another blood sample for cortisol is drawn after 60 minutes.
Treatment of Adrenal Insufficiency
Treatment of adrenal insufficiency is very effective, when done by experienced endocrinologists. Otherwise, there can be serious issues such as quality of life, and increased mortality.1
Drugs to replace adrenal hormones include Prednisone or Hydrocortisone and Florinef.
Is Adrenal Insufficiency Permanent?
Primary adrenal insufficiency – also known as Addison’s disease – is permanent. On the other hand, secondary adrenal insufficiency due to a prolonged use of steroid is reversible once the steroid is discontinued. However, cortisol production takes many months to recover.
Secondary adrenal insufficiency due to a pituitary tumor – and its treatment such as surgery or radiation – is usually permanent.
Actual Case Study of Adrenal Insufficiency
A 38 year old female came to see me complaining of progressive fatigue for several months. She had seen several doctors during this period who told her that everything was fine on her blood tests.
She also developed nausea, vomiting and lost about 30 lbs. in the past two months. Two weeks earlier, she developed vague abdominal pain, prompting surgery for possible appendicitis, but her appendix turned out to be normal on the pathology report.
She denied any fever, chills, night sweats, cough or dysuria. She complained of shortness of breath upon walking only 5-10 feet, but no chest pain.
Past medical history was remarkable for premature ovarian failure at age 27.
Blood pressure=70/40, Pulse= 80, Respiration=24, Temp= 98 F
She appeared sick, malnourished and dehydrated. I admitted her to the hospital for further management.
Serum Sodium = 132, Potassium = 5.5
I made a clinical diagnosis of Addison’s disease and started aggressive intravenous hydration. A cortrosyn stimulation test was performed on admission. Then, she received intravenous hydrocortisone 100 mg every 6 hours.
Within 24 hours, the patient showed remarkable improvement. Her blood pressure came up to 105/60, shortness of breath and anorexia resolved, and fatigue started to improve. Her low serum sodium (Na) and high potassium (K) resolved within 24 hours.
A CT scan of her abdomen showed shrunken adrenal glands.
Three days later, we got the results of cortrosyn stimulation test: baseline serum cortisol severely low as 1 mcg/dl. Cortisol level at 60 minutes – after 0.25 mg of cortrosyn injection – still dangerously low as 1 mcg/dl. This result confirmed the diagnosis of primary adrenal insufficiency or Addison’s disease.
I discharged her home on the following medications: Hydrocortisone 10 mg every morning and 5 mg at lunch time. Florinef 0.075 mg every morning. The patient continues to do well twenty years after her initial diagnosis.
Unfortunately, Addison’s disease (Primary Adrenal Insufficiency) was not suspected in this patient until she came to see me. At that time, she was already in life-threatening condition – Addisonian Crisis. Fortunately, proper treatment – without any further delay – saved her life.
Premature ovarian failure was an important feature in her history that made me highly suspicious of the diagnosis of Addison’s disease.
The important message from this case is that physicians should suspect Addison’s disease in patients with unexplained fatigue especially if they also have other autoimmune diseases such as Hashimoto’s thyroiditis, Type 1 diabetes, pernicious anemia, or premature ovarian failure.
Note: In a severely sick patient, you do not even have to do a cortrosyn stimulation test (we did this test because it was readily available). All you need to do is to draw a serum cortisol level before starting the patient on Hydrocortisone. Under the stress of acute illness, serum cortisol should be more than 25 mcg/dl. A value below this level is consistent with the diagnosis of Addison’s disease in these patients.
Physicians as well as patients should suspect adrenal insufficiency especially in the right clinical settings. Because lack of diagnosis – and treatment – can have horrendous consequences. An experienced endocrinologist is your best bet for the good long-term care of this disease.