From the Guidelines
Addison's disease is a rare endocrine disorder where the adrenal glands don't produce enough cortisol and sometimes aldosterone hormones, and treatment involves hormone replacement therapy, typically with oral hydrocortisone (15-25 mg daily in divided doses) to replace cortisol and sometimes fludrocortisone (0.05-0.2 mg daily) to replace aldosterone, as recommended by the most recent guidelines 1.
Definition and Causes
Addison's disease, also known as primary adrenal insufficiency, occurs when the adrenal cortex is damaged, most commonly by an autoimmune process where the body's immune system attacks its own tissues 2. This damage leads to a deficiency in the production of cortisol and sometimes aldosterone hormones. The disease is rare, affecting approximately 10-15 people per 100,000 population 2.
Symptoms
Symptoms of Addison's disease develop gradually and include:
- Fatigue
- Muscle weakness
- Weight loss
- Low blood pressure
- Darkening of the skin
- Salt cravings
- Low blood sugar
- Nausea
- Diarrhea
- Mood changes
Treatment
Treatment for Addison's disease involves hormone replacement therapy to replace the deficient hormones. The preferred choice of glucocorticoid treatment is hydrocortisone (HC) or cortisone acetate (CA), with no studies showing that one is superior to the other 3. The typical dosage of hydrocortisone is 15-25 mg daily in divided doses, and fludrocortisone is sometimes prescribed at a dose of 0.05-0.2 mg daily to replace aldosterone 4. Patients must take these medications for life and increase their hydrocortisone dose during times of illness, injury, or stress to prevent an adrenal crisis, which is a life-threatening emergency.
Management and Monitoring
Patients with Addison's disease should carry a medical alert card or bracelet and have an emergency injection kit containing hydrocortisone for severe situations 4. Regular monitoring by an endocrinologist is essential to adjust medication doses and prevent complications. The management of glucocorticoids during the peri-operative period is also crucial, and guidelines have been established to ensure the safe management of patients with adrenal insufficiency undergoing surgery or other medical procedures 1, 5.
Quality of Life and Complications
Without treatment, Addison's disease can be fatal, but with proper hormone replacement, patients can lead normal, healthy lives. However, patients with Addison's disease are at risk of developing complications such as osteoporosis, cardiovascular disease, and adrenal crisis, which can be life-threatening if not managed promptly and effectively 2, 1. Regular monitoring and adjustment of medication doses can help prevent these complications and improve the quality of life for patients with Addison's disease.
From the Research
Definition and Causes of Addison's Disease
- Addison's disease, also known as primary adrenal insufficiency, is a rare condition characterized by inadequate hormonal production by the adrenal gland 6.
- The disease is caused by the destruction of the adrenal cortex, leading to a deficiency of cortisol and aldosterone hormones 7, 8.
- Autoimmune adrenalitis is the most common cause of primary adrenal insufficiency in the developed world, where the majority of patients have circulating antibodies against the key steroidogenic enzyme 21-hydroxylase 9.
Symptoms and Diagnosis
- The clinical picture of Addison's disease is caused by deficiency of cortisol and aldosterone, accompanied by adrenal androgen depletion 7.
- Symptoms of acute adrenal crisis include orthostatic hypotension, fever, and hypoglycemia, whereas chronic primary adrenal insufficiency presents with a more insidious history of malaise, anorexia, diarrhea, weight loss, joint, and back pain 8.
- Cutaneous manifestations include darkening of the skin, especially in sun-exposed areas, and hyperpigmentation of the palmar creases, frictional surfaces, vermilion border, recent scars, genital skin, and oral mucosa 8.
- Diagnosis is confirmed by demonstrating inappropriately low cortisol secretion, and elevated plasma levels of adrenocorticotropin and renin 6, 8.
Treatment and Management
- Treatment involves replacement of the deficient hormones, including glucocorticoids and mineralocorticoids 10, 6, 7, 8.
- The current therapy aims to emulate physiological patterns of hormone secretion to achieve well-being and good quality of life 9.
- Patients with Addison's disease require mineralocorticoid replacement, i.e., fludrocortisone, and glucocorticoid replacement, i.e., hydrocortisone or cortisone acetate 10, 7.
- Education of patients and healthcare professionals is essential to minimize the risk of a life-threatening adrenal crisis 9.