Addison’s Disease

Addison’s disease, also known as primary adrenal insufficiency, is a rare, chronic endocrine disorder caused by the insufficient production of cortisol and aldosterone hormones by the adrenal glands. The condition is named after Thomas Addison, who first described it in 1855. Addison’s disease affects approximately 1-2 in every 100,000 people and can occur at any age, although it is most commonly diagnosed in individuals aged 30-50 years.

Pathophysiology:
In most cases, Addison’s disease occurs as a result of an autoimmune process that damages the adrenal cortex, leading to the impaired production of cortisol and aldosterone hormones. Other causes of Addison’s disease include infections, such as tuberculosis or HIV, or damage to the adrenal glands from surgery or radiation therapy.

Signs and symptoms:
The signs and symptoms of Addison’s disease can vary, and they typically develop gradually over several months or years. Some common signs and symptoms include:

Fatigue and weakness
Weight loss and decreased appetite
Darkening of the skin (hyperpigmentation)
Low blood pressure
Dizziness or fainting
Salt cravings
Muscle or joint pain
Nausea, vomiting, or diarrhea
Depression or anxiety
Investigation:
The diagnosis of Addison’s disease involves a series of tests, including blood tests to measure cortisol and aldosterone levels, as well as tests to assess the function of the adrenal glands, such as an ACTH stimulation test or a CRH stimulation test. Imaging tests, such as a CT scan or MRI, may also be done to evaluate the adrenal glands for any structural abnormalities.

Treatment:
The treatment of Addison’s disease involves lifelong hormone replacement therapy with cortisol and aldosterone. The medications used for hormone replacement include hydrocortisone, prednisone, or dexamethasone for cortisol replacement and fludrocortisone for aldosterone replacement. The dosage of these medications may need to be adjusted based on the individual’s response to treatment, and regular follow-up with an endocrinologist is necessary.

Dos and Don’ts:
Individuals with Addison’s disease should wear a medical alert bracelet or necklace at all times, indicating their condition and the medications they are taking. They should also carry an emergency kit containing an injectable form of cortisol (hydrocortisone) in case of a medical emergency or illness. Additionally, individuals with Addison’s disease should avoid stressful situations whenever possible, as stress can trigger a sudden worsening of symptoms.

References:

Nieman LK. Addison’s disease. In: Jameson JL, De Groot LJ, editors. Endocrinology: Adult and Pediatric. 7th ed. Philadelphia, PA: Elsevier Saunders; 2016. p. 1904-1918.
Husebye ES, Allolio B, Arlt W, Badenhoop K, Bensing S, Betterle C, et al. Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency. J Intern Med. 2014 Feb;275(2):104-15.
National Institute of Diabetes and Digestive and Kidney Diseases. Addison’s Disease. Accessed on March 25, 2023. https://www.niddk.nih.gov/health-information/endocrine-diseases/addisons-disease

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