From the Guidelines
Adrenal insufficiency (Addison's disease) typically manifests between ages 30-50, with autoimmune causes being most common in adults. This age range is supported by the fact that primary adrenal insufficiency affects 1 in 8–10,000 children, but the incidence increases with age, suggesting that the disease often develops later in life 1.
Key Points to Consider
- The prevalence of adrenal insufficiency in adult and paediatric populations varies, with primary adrenal insufficiency being more common in adults 1.
- Autoimmune causes are the most common cause of primary adrenal insufficiency in adults, which tends to develop between ages 30-50 1.
- Secondary adrenal insufficiency often appears in middle adulthood, frequently due to prolonged corticosteroid use or pituitary disorders, but can occur at any age 1.
- Congenital adrenal hyperplasia, a genetic form of adrenal insufficiency, is present from birth and typically diagnosed in infancy or early childhood, affecting 1 in 15,000 live births 1.
Treatment and Management
Treatment for adrenal insufficiency generally involves hormone replacement therapy with glucocorticoids and sometimes mineralocorticoids, with patients requiring lifelong treatment and dose adjustments during illness or stress 1.
Importance of Early Diagnosis
Early diagnosis is crucial as untreated adrenal insufficiency can be fatal, particularly during times of physiological stress, highlighting the need for prompt recognition and management of the condition 1.
From the Research
Age of Manifestation
There is no specific information in the provided studies regarding the typical age at which adrenal insufficiency (Addison's disease) manifests.
General Information about Adrenal Insufficiency
- Adrenal insufficiency is characterized by inadequate hormonal production by the adrenal gland, which could be primary or secondary/tertiary 2.
- The diagnosis is often delayed by months, and many patients present with acute adrenal crisis 2.
- Sudden withdrawal from chronic glucocorticoid therapy is the most common cause of adrenal insufficiency 3, 2.
- Primary adrenal insufficiency is a relatively rare condition, and most medical specialists will only manage a handful of these patients in their career 2.
Diagnosis and Treatment
- The diagnosis of adrenal insufficiency is made by demonstrating low basal and/or stimulated serum cortisol and should be followed by appropriate investigations to establish the underlying aetiology 3.
- Maintenance glucocorticoid replacement is usually given as a twice or thrice daily hydrocortisone preparation 3, 4.
- Patients with primary adrenal insufficiency also require mineralocorticoid replacement 3, 4.
- Regular monitoring for features of under- and over-replacement is essential during follow-up 3.
Case Reports
- A 40-year-old male presented with asymptomatic, slowly progressive skin hyperpigmentation, fatigue, and weight loss, and was diagnosed with primary adrenal insufficiency (Addison's disease) 5.