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

...gnosis...

...ould Be Tested and How...

...Endocrine Society (ES) recommends diagnostic...

...s confirmatory testing with the corticotrop...


...mal Diagnostic Te...

...the standard dose (250 μg for adults and children...

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

...f a corticotropin stimulation test is not feasi...

...ecommends measurement of plasma ACTH t...

...ends the simultaneous measurement of plasma r...

...sts that the etiology of PAI should be determi...


...1. Clinical Features of Adrenal Insufficienc...


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


...2. Major Etiologies of PAI and Associ...


Treatment

...reatmen...

Treatment of Primary Adrenal Insufficie...

...th severe adrenal insufficiency symptoms or adre...


...cocorticoid Replacement Regim...

...recommends glucocorticoid therapy in all patient...

...suggests using hydrocortisone (15–25 mg...

...alternative to hydrocortisone, ES suggests using p...

...against using dexamethasone for the...

...suggests monitoring glucocorticoid...

...ts against hormonal monitoring of glu...


...neralocorticoid Replacement in PAI...

...commends that all patients with confirmed aldoste...

...s monitoring mineralocorticoid replacement primar...

...who develop hypertension while receiving flud...

...od pressure remains uncontrolled, ES suggests init...


...epiandrosterone Replacement

...gests a trial of dehydroepiandrosterone (...

...s an initial period of 6 months of DHEA...

...S suggests monitoring DHEA replacement by m...


...tment During Pregnancy...

...s that pregnant patients with PAI be monitored f...

...hat, based on the individual clinical cour...

...gnant women with PAI, ES suggests usin...

...nd recommends against using dexamethasone bec...

...mends hydrocortisone stress dosing during the acti...


...ment and Monitoring During Childh...

...en with PAI, ES suggests treatment with hy...

...children with PAI, ES suggests avoidin...

...ts monitoring glucocorticoid repla...

...h PAI and confirmed aldosterone defic...


...agement and Prevention of Adrenal Crisis in Pat...

...ds that patients with suspected ad...

If hydrocortisone is unavailable, ES suggests...

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

...uggests patient education concerning g...

...S recommends that all patients should b...

...ommends that every patient should be equip...


Additional Monitoring Requireme...

...that adults and children with PAI...

...at PAI patients be evaluated annually for sym...

...s periodic screening for autoimmune diseases known...

...sts patient education about increasi...

...genetic counseling for patients with PAI due to...


...le 3. Management of PAI in Specific Situati...


...le 4. Measures for Prevention of Adrenal Cr...