Primary Adrenal Insufficiency
Key Points
Key Points
- Primary adrenal insufficiency (PAI) was first described in 1855 by Thomas Addison and is therefore commonly termed Addison’s disease.
- PAI is defined by the inability of the adrenal cortex to produce sufficient amounts of glucocorticoids and/or mineralocorticoids.
- PAI is a severe and potentially life-threatening condition due to the central role of these hormones in energy, salt, and fluid homeostasis.
- Cortisol deficiency results in a decrease in feedback to the hypothalamic-pituitary axis and subsequent enhanced stimulation of the adrenal cortex by elevated levels of plasma ACTH. Consequent to disruption of adrenal mineralocorticoid synthesis, renin release by the juxtaglomerular cells of the kidneys increases. This is of clinical, diagnostic, and therapeutic relevance because PAI needs to be distinguished from secondary adrenocortical insufficiency due to insufficient production of ACTH and without impact on the renin-angiotensin-aldosterone system.
Diagnosis
Diagnos...
...ould Be Tested and How...
...ociety (ES) recommends diagnostic testi...
...onfirmatory testing with the corticotropin stimul...
...ptimal Diagnostic T...
...sts the standard dose (250 μg for adults...
...suggests the low-dose (1 μg) corticotrop...
...a corticotropin stimulation test is not...
ES recommends measurement of plasm...
ES recommends the simultaneous measurem...
ES suggests that the etiology of PAI should be...
...able 1. Clinical Features of Adren...
...agnostic approach to the patient with PAI...
...r Etiologies of PAI and Associated Features...
Treatment
...atment...
...of Primary Adrenal Insufficiency in Adults...
...s with severe adrenal insufficiency sympt...
...rticoid Replacement Regimen...
...glucocorticoid therapy in all patients wi...
...ests using hydrocortisone (15–25...
...tive to hydrocortisone, ES suggests usi...
...ainst using dexamethasone for the t...
...itoring glucocorticoid replacement using clin...
...S suggests against hormonal monitoring of gluco...
...ticoid Replacement in PAI...
...mends that all patients with confirmed aldo...
ES recommends monitoring mineralocorticoid replace...
...s who develop hypertension while receivi...
...f blood pressure remains uncontrolled, ES suggests...
...oepiandrosterone Replacemen...
...suggests a trial of dehydroepiandr...
...ggests an initial period of 6 months...
...ggests monitoring DHEA replacement by m...
...reatment During Pregnan...
ES suggests that pregnant patients with...
...gests that, based on the individual clinical...
...nt women with PAI, ES suggests using hydrocortis...
and recommends against using dexamethas...
...mmends hydrocortisone stress dosing during the...
...and Monitoring During Childhood
...ildren with PAI, ES suggests treatme...
...hildren with PAI, ES suggests avoiding synthetic,...
...s monitoring glucocorticoid replacement by...
...n with PAI and confirmed aldosterone deficiency,...
...ment and Prevention of Adrenal Crisis in Patient...
...ommends that patients with suspected adr...
...rocortisone is unavailable, ES sugg...
...prevention of adrenal crisis, ES suggest...
...suggests patient education concerning glucocor...
...s that all patients should be equipped with a ster...
...commends that every patient should be equipped wi...
...ditional Monitoring Requ...
...uggests that adults and children with...
...ggests that PAI patients be evaluated annual...
...ts periodic screening for autoimmune d...
...sts patient education about increasing the dosa...
...S suggests genetic counseling for pa...
...agement of PAI in Specific SituationsHaving troubl...
...res for Prevention of Adrenal CrisisHaving trouble...