Addison’s disease is also called as chronic adrenal insufficiency, hypocortisolism or hypocorticism. It is a rare disorder where the adrenal glands are not able to produce glucocorticoids and less often mineralocorticoids in sufficient quantity. The condition was first described by Dr Thomas Addison, the British physician who first described the condition.
Causes of Addison disease
Adrenal dysgenesis
This means adrenal glands which produce glucocorticoid are absent.
Impaired Formation of Hormone
To form cortisol, the adrenal gland requires cholesterol, which is then converted biochemically into steroid hormones. Interruptions in the delivery of cholesterol include Smith-Lemli-Opitz syndrome and abetalipoproteinemia.
Congenital adrenal hyperplasia may also cause impaired synthesis
Medications
Medicationslike ketoconazole interfere with enzymes that synthesize the hormone, while others like rifampicin, phenytoin increase hormonal breakdown.
Adrenal destruction
- Autoimmune adrenalitis
- Adrenoleukodystrophy (ALD)
- Metastasis (
- Hemorrhage (Waterhouse-Friderichsen syndrome or antiphospholipid syndrome)
- Infection
- Amyloidosis.
What Are Symptoms and signs Of Addison Disease
The disease symptoms are insidious in onset and per se the symptoms are quite non specific. Following symptoms can be noted with this condtion.
- Fatigue
- Lightheadedness upon standing or while upright
- Muscle weakness
- Weight loss
- Anxiety
- Nausea, vomiting, diarrhea
- Headache, sweating
- Changes in mood and personality
- Vague joint and muscle pains.
- Marked cravings for salt or salty foods [Due to loss of salt in urine]
- Darkening of the skin [Due to hypepigmentation]
- Orthostatic hypotension [Low blood pressure that falls further when standing]
Routine Lab Investigations may show
- Hypercalcemia or low calcium level
- Hypoglycemia or low blood sugar level
- Hyponatraemia or low blood sodium levels
- Hyperkalemia or raised blood potassium levels
- Eosinophilia and lymphocytosis
- Metabolic acidosis (increased blood acidity), also due to loss of the hormone aldosterone
Special Tests
ACTH stimulation test
Low adrenal hormone levels even after appropriate stimulation with synthetic pituitary ACTH hormone tetracosactide
Renin and adrenocorticotropic hormone levels, ultrasound, computed tomography or magnetic resonance imaging are other tests used in making a diagnosis.
Treatment
Treatmentaims at replacing the missing cortisol. Hydrocortisone or prednisone tablets are commonly used and are continued for life. In addition, many patients require fludrocortisone as replacement for the missing aldosterone.
