In Addison’s disease (synonyms: Addison’s disease; Addison’s melanosis; Addison’s syndrome; bronchial skin disease; bronchial disease; suprarenal melasma; primary adrenal insufficiency; primary adrenocortical insufficiency; primary adrenocortical atrophy; primary adrenocortical insufficiency; primary adrenocortical hypofunction; primary hypoadrenalism; primary hypoadrenocorticism; primary suprarenal insufficiency; ICD-10-GM E27. 1: Primary adrenocortical insufficiency: Addison’s disease) is primary adrenocortical insufficiency (NNR insufficiency; adrenocortical insufficiency). This results in decreased secretion of all adrenocortical hormones (NNR hormones). The disease (NNI) is the result of a progressive destruction of the adrenal cortex, which must affect more than 90%, because only then clinical symptoms of adrenal insufficiency appear.
There are many causes of primary adrenocortical insufficiency:
- Genetic causes (frequency: very rare).
- Adrenoleukodystrophy (synonyms: X-ALD; Addison-Schilder syndrome) – X-linked recessive disorder leading to a defect in steroid hormone synthesis with accumulation of overlong-chain fatty acids in the NNR and CNS; consequently, neurological deficits and dementia develop with onset in childhood.
- Familial glucocorticoid deficiency (isolated cortisol deficiency with normal aldosterone concentrations; e.g., due to ACTH receptor defect (→ lack of responsiveness of ACTH to NNR).
- Primary cortisol resistance (due toabsent or defective glucocorticoid receptors at target tissue (→ secondary hypersecretion of: ACTH, cortisol, androgens and mineralocorticoids).
- Autoimmune destruction of the NNR (75% of cases).
- Infections (10-25% of cases)
- Vascular (vascular) causes.
- Malignancies (tumor diseases; metastases in the NNR).
Sex ratio: women are more commonly affected than men.
Frequency peak: the average age of onset at diagnosis is 40 years.
Addison’s disease occurs relatively rarely.
The prevalence (disease incidence) is 10-11 cases per 100,000 population per year.
The incidence (frequency of new cases) is about 5 cases per 1,000,000 inhabitants per year (in Germany). Course and prognosis: Because of mostly unspecific clinical symptoms, the diagnosis is often delayed! The disease is potentially life-threatening. Complete loss of adrenal function occurs. The process can be acute or chronic insidious. The missing hormones must be substituted for life. Addisonian crisis (vigilance disorder (impaired consciousness), dehydration (lack of fluids), fever, hypoglycemia (low blood sugar)) is the acute life-threatening form of the process and requires emergency hospital treatment. Sufferers should always carry an ID card informing them of their condition in case of emergency.
Comorbidities (concomitant diseases): Addison’s disease is associated with other autoimmune diseases in more than half of cases, autoimmune thyroiditis (AIT) in 50%, and type 1 diabetes mellitus in 10-15%.
