Addison Disease: Causes, Symptoms and Treatment
Addison disease is a rare disorder of the adrenal cortex in which too little cortisol and aldosterone are produced. It requires lifelong hormone replacement therapy.
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Addison disease is a rare disorder of the adrenal cortex in which too little cortisol and aldosterone are produced. It requires lifelong hormone replacement therapy.
What is Addison Disease?
Addison disease (also known as primary adrenal insufficiency) is a rare, chronic condition in which the adrenal cortex does not produce sufficient amounts of vital hormones. The two main hormones affected are cortisol (a stress hormone) and aldosterone (which regulates fluid and salt balance). The disease was first described in 1855 by British physician Thomas Addison.
Causes
The most common cause of Addison disease is an autoimmune reaction, in which the immune system mistakenly attacks and destroys adrenal cortex tissue. Other possible causes include:
- Tuberculosis – once the leading cause, still relevant in developing countries
- Other infections (e.g., HIV, fungal infections)
- Metastases (cancer spread) to the adrenal glands
- Surgery or injury to the adrenal glands
- Genetic disorders (e.g., adrenoleukodystrophy)
Symptoms
The symptoms of Addison disease usually develop gradually and are often non-specific in the early stages. Common complaints include:
- Persistent fatigue and exhaustion
- Weight loss and reduced appetite
- Low blood pressure (hypotension), especially when standing up
- Brownish discoloration of the skin and mucous membranes (hyperpigmentation)
- Nausea, vomiting, and abdominal pain
- Salt cravings (strong desire for salty foods)
- Muscle and joint pain
- Depressive moods and irritability
Addisonian Crisis
A life-threatening complication is the so-called Addisonian crisis (acute adrenal insufficiency). It can be triggered by physical stress such as infections or surgery and is characterized by a severe drop in blood pressure, loss of consciousness, and circulatory failure. Immediate emergency medical treatment is essential in this situation.
Diagnosis
The diagnosis is confirmed through several investigations:
- Blood tests: Measurement of cortisol and ACTH levels in the blood
- ACTH stimulation test: The gold standard in diagnostics – synthetic ACTH is injected; in Addison disease, cortisol levels do not rise adequately
- Electrolytes: Detection of low sodium and elevated potassium levels in the blood
- Imaging: CT or MRI of the adrenal glands to identify the underlying cause
- Autoantibody tests: Detection of antibodies against adrenal cortex tissue
Treatment
Addison disease cannot be cured, but it is very manageable with the right treatment. Therapy consists of lifelong hormone replacement:
- Hydrocortisone (cortisol replacement): Typically taken 2–3 times daily to mimic the natural daily cortisol rhythm
- Fludrocortisone (aldosterone replacement): Used to regulate fluid and salt balance
Dose Adjustment During Stress
During physical stress (fever, surgery, serious illness), the hydrocortisone dose must be temporarily increased to prevent an Addisonian crisis. Patients are generally provided with an emergency card and an emergency injection kit containing hydrocortisone for self-administration.
Lifestyle
With the correct treatment, most patients can lead a largely normal life. Regular medical check-ups, adequate salt intake, and carrying an emergency identification card are important accompanying measures.
References
- Bornstein SR et al. – Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline. Journal of Clinical Endocrinology and Metabolism, 2016.
- Arlt W, Allolio B – Adrenal insufficiency. The Lancet, 2003; 361(9372): 1881–1893.
- World Health Organization (WHO) – International Classification of Diseases (ICD-11), Addison Disease entry, 2022.
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Related search terms: Addison Disease + Addison´s Disease + Addison Syndrome + Morbus Addison