Addison's disease
Background
Types of adrenal insufficiency (primary, secondary, and tertiary) compared to normal physiology. In its normal state, the hypothalamus secretes corticotropin-releasing hormone (CRH) to induce the pituitary gland to secrete adrenocorticotropic hormone (ACTH) to induce the adrenal gland to secrete cortisol. Cortisol then exerts negative feedback by inhibiting the secretion of CRH and ACTH in the hypothalamus and pituitary gland, respectively.
- Primary adrenal insufficiency
- Autoimmune disease (70-90%)
- Tuberculosis previously most common cause
- Other causes include infectious, metastatic cancer, lymphoma, adrenal hemorrhage, infarction, drugs
- Typically presents in adults between 30 and 50 years of age
- All ages, races, genders can be affected
- Higher incidence with other autoimmune deficiencies
Clinical Features
- Abdominal pain
- Weakness and fatigue
- Salt craving
- Abnormal skin pigmentation - will often look patchy
- Addisonian crisis:
- Hypotension
- Neurologic symptoms: coma
Differential Diagnosis
- Corticosteroid withdrawal
- Secondary adrenal insufficiency - deficiency of ACTH (produced by the pituitary gland)
- Tertiary adrenal insufficiency - deficiency of CRH (produced by the hypothalamus)
Adrenal crisis
Evaluation
Laboratory findings
- Hypercalcemia
- Hypoglycemia
- Hyponatremia
- Hyperkalemia
- Metabolic acidosis
- Low stimulation of the renal distal tubule by aldosterone = sodium wasting in the urine and H+ retention
Evaluation
- Chemistry
- Random cortisol, renin, and ACTH levels
- Do not wait for levels before starting treatment
- ACTH (cosyntropin) stimulation test
Management
- Hormone Replacement[1]
- PO hydrocortisone and fludrocortisone
- Adrenal crisis should be treated with 100mg hydrocortisone IV followed by 50mg IV q6 hours
Disposition
- New diagnosis: strong consideration for admission
- Crisis?: Consider admission based on electrolytes and clinical presentation
See Also
External Links
References
- ↑ de Herder WW, van der Lely AJ (May 2003). "Addisonian crisis and relative adrenal failure". Reviews in Endocrine and Metabolic Disorders 4 (2): 143–7.