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Morbus Addison: Causes, Symptoms and Treatment

Morbus Addison is a rare disorder of the adrenal cortex in which too few vital hormones are produced. It requires lifelong hormone replacement therapy.

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Things worth knowing about "Morbus Addison"

Morbus Addison is a rare disorder of the adrenal cortex in which too few vital hormones are produced. It requires lifelong hormone replacement therapy.

What is Morbus Addison?

Morbus Addison (also known as primary adrenal insufficiency) is a rare but serious condition in which the adrenal cortex does not produce sufficient hormones. The hormones primarily affected are cortisol and aldosterone, which are essential for vital body functions such as metabolism, blood pressure regulation, and immune defence. The disease is named after the British physician Thomas Addison, who first described it in the 19th century.

Causes

In the majority of cases, Morbus Addison is caused by an autoimmune reaction in which the body's own immune system mistakenly attacks and destroys the adrenal cortex. Other possible causes include:

Infections, particularly tuberculosis (a common cause worldwide)
Metastases from other cancers spreading to the adrenal glands
Certain medications that inhibit cortisol metabolism
Bleeding or impaired blood supply to the adrenal glands
Genetic conditions such as congenital adrenal hyperplasia

Symptoms

Symptoms of Morbus Addison usually develop gradually over weeks or months. Typical complaints include:

Chronic fatigue and general weakness
Unintentional weight loss and loss of appetite
Low blood pressure (hypotension), especially when standing up (orthostatic hypotension)
Darkening of the skin and mucous membranes (hyperpigmentation), especially on palms, joints, and scars
Salt craving (strong desire for salty foods)
Nausea, vomiting, and abdominal pain
Psychological changes such as depression or irritability

Addisonian Crisis

An Addisonian crisis (adrenal crisis) is a life-threatening emergency triggered by acute, severe cortisol deficiency, for example caused by infections, surgery, or extreme stress. Symptoms include a sudden and severe drop in blood pressure, vomiting, intense abdominal pain, and loss of consciousness. Immediate emergency treatment with intravenous cortisol is life-saving.

Diagnosis

The diagnosis of Morbus Addison requires several investigations:

Blood test: Measurement of morning cortisol levels and ACTH (adrenocorticotropic hormone from the pituitary gland)
ACTH stimulation test: An artificial ACTH stimulus reveals whether the adrenal glands can secrete sufficient cortisol
Detection of autoantibodies against adrenal cortex tissue in the blood
Imaging methods such as CT or MRI of the adrenal glands to determine the underlying cause
Measurement of electrolytes (sodium, potassium) in the blood

Treatment

Morbus Addison cannot be cured, but it is very well manageable with lifelong hormone replacement therapy. Treatment includes:

Hydrocortisone (a synthetic form of cortisol) to replace the missing cortisol, usually taken 2 to 3 times daily
Fludrocortisone to replace missing aldosterone and regulate the salt-water balance
During stressful situations (illness, surgery), the cortisol dose must be adjusted (increased) under medical guidance (sick-day rules)
An emergency card and an emergency injection of hydrocortisone in case of an Addisonian crisis

With the correct treatment, affected individuals can lead a largely normal life. However, regular medical check-ups are essential.

References

Bornstein SR et al. - Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline. Journal of Clinical Endocrinology & Metabolism, 2016.
Erichsen MM et al. - Addison's disease in Europe. Best Practice & Research Clinical Endocrinology & Metabolism, 2009.
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) - Adrenal Insufficiency and Addison's Disease. Available at: www.niddk.nih.gov

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