Primary Adrenal Insufficiency

Publication Date: February 1, 2016

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

Diagnosi...

...Should Be Tested and How

...Endocrine Society (ES) recommends diagn...

...S recommends confirmatory testing with the c...


...Diagnostic Tests...

...standard dose (250 μg for adults a...

...suggests the low-dose (1 μg) corticotropin test...

...a corticotropin stimulation test is not...

...ecommends measurement of plasma ACTH to es...

...e simultaneous measurement of plasma renin and...

...t the etiology of PAI should be deter...


...le 1. Clinical Features of Adrenal Insufficien...


.... Diagnostic approach to the patient with PAI...


...e 2. Major Etiologies of PAI and Associated Fe...


Treatment

...atment

...of Primary Adrenal Insufficiency in Adults...

...tients with severe adrenal insufficiency sym...


...rticoid Replacement Regimen...

...ucocorticoid therapy in all patients with confirm...

...suggests using hydrocortisone (15–25 mg) or co...

...ternative to hydrocortisone, ES suggests us...

...inst using dexamethasone for the treatment of...

...suggests monitoring glucocorticoid repla...

...ainst hormonal monitoring of glucocortico...


...alocorticoid Replacement in PAI...

...that all patients with confirmed aldosterone def...

...ecommends monitoring mineralocorticoid repl...

...who develop hypertension while receivin...

...blood pressure remains uncontrolled, ES su...


...iandrosterone Replacement...

...s a trial of dehydroepiandrosterone (DHEA) replace...

...suggests an initial period of 6 months of DHEA r...

...s monitoring DHEA replacement by mea...


...nt During Pregnancy...

...uggests that pregnant patients with PAI be monito...

...s that, based on the individual cl...

...egnant women with PAI, ES suggests using hyd...

...mends against using dexamethasone because it...

...hydrocortisone stress dosing during the active...


...and Monitoring During Childhood...

...PAI, ES suggests treatment with hydrocorti...

...th PAI, ES suggests avoiding syntheti...

...gests monitoring glucocorticoid rep...

...children with PAI and confirmed aldosterone defic...


...Prevention of Adrenal Crisis in Patients w...

...at patients with suspected adrenal crisis...

...rocortisone is unavailable, ES suggests prednis...

...vention of adrenal crisis, ES suggests ad...

...S suggests patient education concern...

...at all patients should be equipped wit...

...ends that every patient should be equipped...


...itional Monitoring Requirement...

...suggests that adults and children with PA...

...uggests that PAI patients be evaluated a...

ES suggests periodic screening for autoi...

...ient education about increasing the do...

...netic counseling for patients with PAI du...


...nagement of PAI in Specific SituationsHaving t...


...s for Prevention of Adrenal CrisisHaving tro...