Introduction
Adrenal insufficiency is a rare endocrine (or hormonal) disorder that occurs when the adrenal glands cease to produce any or not enough glucocorticoids (the hormone cortisol or cortisone) and, in some cases, mineralocorticoids (the hormone aldosterone) and androgens. There are different types of adrenal insufficiency, only one of which is Addison’s disease.
Cortisol, produced in the adrenal glands, is a steroid hormone which is essential for a wide range of processes throughout the body including metabolism and the immune response. It also has a very important role in helping the body respond to stress. Failure of the adrenal glands therefore requires lifelong, continuous cortisol replacement therapy.
Adrenal insufficiency occurs in all age groups and afflicts men and women alike. Among one million people, between 82-144 are diagnosed with primary and 150-280 with secondary adrenal insufficiency.
If a sudden emotional or physical stress (e.g. an accident/trauma or fever/infection) occurs, the disease can, despite correct and constant replacement therapy, quickly progress to a condition called an Addisonian crisis, adrenal crisis or acute adrenal insufficiency. This is a potentially life-threatening situation and requires immediate emergency treatment.
The onset of symptoms is often very gradual and may include fatigue, dizziness, weight loss, low blood pressure, muscle weakness, mood changes and sometimes (in primary adrenal insufficiency only) darkening of the skin in both sun-exposed and non-exposed parts of the body.
Because the condition is rare and presents a broad range of nonspecific symptoms its diagnosis is often delayed. Once diagnosed, people should consult an endocrinologist (specialist for hormonal diseases) as adrenal insufficiency can be associated with other hormone deficiencies.
In this chapter: The Adrenal Glands | What is Cortisol? | The regulation of Cortisol Release (HPA Axis) | What is Aldosterone?
2. Types of Adrenal Insufficiency
In this chapter: Primary Adrenal Insufficiency (one type of which is Addison’s Disease) | Secondary Adrenal Insufficiency | Tertiary Adrenal Insufficiency | Comparison of primary, secondary and tertiary AI
3. Symptoms
In this chapter: Lab Tests | Imaging Tests
5. Treatment of Adrenal Insufficiency
In this chapter: Cortisol replacement | Aldosterone replacement | Androgen replacement
6. Stress dosing and Sick day Management
In this chapter: Guidelines for stress and sick day dosing | Surgery and Special Diagnostic Procedures (planned and emergency) | Pregnancy
7. Acute Adrenal Insufficiency, Addisonian Crisis or Adrenal Crisis
In this chapter: Incidence Symptoms of Adrenal Crisis | Causes and Development | Treatment | Reducing the Risk of Adrenal Crises: Knowledge is Key
9. Other Conditions and Drug Interactions
In this chapter: Drug interactions | Autoimmune Diseases / Polyendocrine Deficiency Syndrome | Steroid Therapy and Adrenal Suppression
10. Long-term Management: Educate yourself!
In this chapter: Seek the help of a specialist | Be your own advocate | Adrenal Crisis: Don’t stress, but be prepared
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> CHAPTER OVERVIEW
Anatomy/Physiology | Types of Adrenal Insufficiency | Symptoms | Testing/Diagnosis | Treatment | Stress dosing/Sick day Management | Adrenal Crisis | Quality of Life and Risks | Other Conditions and Drugs | Long-term Management | Suggested Reading | Literature/References
Author: Gisela Spallek, MD PhD
Edited by Maria Stewart, Director of AIC and deputy editor Des Rolph, Associate Director of AIC
This document is based on an extensive study and review of current clinical research on adrenal insufficiency. The author is a physician with over 20 years experience of presenting medical information and data in an easy to understand format for the layperson, see spallek.com for more details.
Last update: 05 June 2020