Primary Adrenal Insufficiency
Key Points
Key Points
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
...Diagnosis...
...Who Should Be Tested an...
...ciety (ES) recommends diagnostic testing to e...
...confirmatory testing with the corticot...
...Optimal Di...
...uggests the standard dose (250 μg...
...gests the low-dose (1 μg) corticotropin test fo...
...ropin stimulation test is not feasible, ES sugge...
...ecommends measurement of plasma ACTH to e...
...s the simultaneous measurement of plas...
...sts that the etiology of PAI should b...
...e 1. Clinical Features of Adrenal Insuf...
...1. Diagnostic approach to the patient with...
...or Etiologies of PAI and Associated Feat...
Treatment
...Tre...
...s with severe adrenal insufficiency symptoms o...
...Glucocorticoid Replac...
...ucocorticoid therapy in all patients...
ES suggests using hydrocortisone (...
...native to hydrocortisone, ES suggests usin...
...sts against using dexamethasone for t...
ES suggests monitoring glucocortico...
...gests against hormonal monitoring...
...Mi...
...recommends that all patients with confirmed al...
...recommends monitoring mineralocorticoid replacem...
...develop hypertension while receiving f...
...pressure remains uncontrolled, ES suggests in...
...ts a trial of dehydroepiandrosterone (DHEA) r...
ES suggests an initial period of 6 months of...
ES suggests monitoring DHEA replacement b...
...Treatment During...
...that pregnant patients with PAI be monitored for...
..., based on the individual clinical course,...
...nt women with PAI, ES suggests using hydrocortiso...
...nd recommends against using dexamethasone beca...
...drocortisone stress dosing during the active p...
...Treatment and Moni...
...PAI, ES suggests treatment with hydrocortis...
...ldren with PAI, ES suggests avoiding synthe...
...toring glucocorticoid replacement by...
...h PAI and confirmed aldosterone defic...
...Management and Prevention o...
...mends that patients with suspected adrenal crisis...
...ocortisone is unavailable, ES suggest...
...the prevention of adrenal crisis, ES suggests...
...suggests patient education concerning gluco...
...mends that all patients should be equi...
...at every patient should be equipped wit...
...Additional Monitor...
...S suggests that adults and children...
...S suggests that PAI patients be evaluated annua...
...riodic screening for autoimmune diseases known to...
...S suggests patient education about increas...
...genetic counseling for patients with PAI du...
...gement of PAI in Specific Situations...
...s for Prevention of Adrenal Crisis...