Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline

Abstract

We recommend diagnostic tests for the exclusion of primary adrenal insufficiency in all patients with indicative clinical symptoms or signs. In particular, we suggest a low diagnostic (and therapeutic) threshold in acutely ill patients, as well as in patients with predisposing factors. This is also recommended for pregnant women with unexplained persistent nausea, fatigue, and hypotension. We recommend a short corticotropin test (250 μg) as the "gold standard" diagnostic tool to establish the diagnosis. If a short corticotropin test is not possible in the first instance, we recommend an initial screening procedure comprising the measurement of morning plasma ACTH and cortisol levels. Diagnosis of the underlying cause should include a validated assay of autoantibodies against 21-hydroxylase. In autoantibody-negative individuals, other causes should be sought. We recommend once-daily fludrocortisone (median, 0.1 mg) and hydrocortisone (15-25 mg/d) or cortisone acetate replacement (20-35 mg/d) applied in two to three daily doses in adults. In children, hydrocortisone (∼8 mg/m(2)/d) is recommended. Patients should be educated about stress dosing and equipped with a steroid card and glucocorticoid preparation for parenteral emergency administration. Follow-up should aim at monitoring appropriate dosing of corticosteroids and associated autoimmune diseases, particularly autoimmune thyroid disease.

Guidelines for the diagnosis and management of critical illness-related corticosteroid insufficiency (CIRCI) in critically ill patients (Part I): Society of Critical Care Medicine (SCCM) and European Society of Intensive Care Medicine (ESICM) 2017

“…We found one single-center randomized trial that compared low-dose ACTH (1 µg) stimulation test with total random cortisol for diagnosis of adrenal insufficiency in 59 adults with septic shock [10]. Compared with total random cortisol, the low-dose ACTH test was better able to predict a longer duration of vasopressor requirement and hemodynamic response to corticosteroids.…”

Nebenniereninsuffizienz und Nebenniereninzidentalome

“…Eine diagnostische Abklärung auf eine primäre Nebenniereninsuffizienz wird bei akut kranken PatientInnen empfohlen, die an anderweitig nicht erklärbaren Symptome/Beschwerden leiden, welche als suggestiv für eine primäre Nebenniereninsuffizienz angesehen werden: Volumenmangel, Hypotonie,Hyponatriämie,Hyperkaliämie,Fieber,abdominelle Schmerzen,Hyperpigmentierung, oder, speziell bei Kindern, Hypoglykämie [1]. Bei ausgeprägter Symptomatik sollte jedoch schon bei Verdacht auf eine Nebenniereninsuffizienz (Verdacht auf Addison Krise) eine entsprechende Therapie (z.…”

Skeletal fragility induced by overtreatment of adrenal insufficiency

“…Unfortunately, we as treating physicians have a tendency to give supraphysiological glucocorticoid doses to minimize the risk of adrenal crisis in AD and CAH, but also to suppress adrenal androgens in the latter [4,10,11]. However, from a 2-year prospective study it was recently reported that a reduction of the glucocorticoid doses in AD and CAH led to improved BMD without increased risk of adrenal crisis while dose increments decreased BMD [12].…”