Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline.
By S. R. Bornstein, B. Allolio, W. Arlt, A. Barthel, A. Don-Wauchope, G. Hammer, Eystein S. Husebye, D. Merke, M. H. Murad, Constantine A. Stratakis and 4 more. Journal of Clinical Endocrinology and Metabolism, 2016
1,483 citations recorded by the source corpus.
OBJECTIVE This clinical practice guideline addresses the diagnosis and treatment of primary adrenal insufficiency. PARTICIPANTS The Task Force included a chair, selected by The Clinical Guidelines Subcommittee of the Endocrine Society, eight additional clinicians experienced with the disease, a methodologist, and a medical writer. The co-sponsoring associations (European Society of Endocrinology and the American Association for Clinical Chemistry) had participating members. The Task Force received no corporate funding or remuneration in connection with this review. EVIDENCE This evidence-based guideline was developed using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system to determine the strength of recommendations and the quality of evidence. CONSENSUS PROCESS The evidence used to formulate recommendations was derived from two commissioned systematic reviews as well as other published systematic reviews and studies identified by the Task Force. The guideline was reviewed and approved sequentially by the Endocrine Society's Clinical Guidelines Subcommittee and Clinical Affairs Core Committee, members responding to a web posting, and the Endocrine Society Council. At each stage, the Task Force incorporated changes in response to written comments. CONCLUSIONS 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.
Subfields: Endocrinology, Diabetes and Metabolism, Molecular Biology
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Citations in the corpus
- Management of Immune-Related Adverse Events in Patients Treated With Immune Checkpoint Inhibitor Therapy: American Society of Clinical Oncology Clinical Practice Guideline (2018, background): “Fludrocortisone is needed in addition to hydrocortisone in most cases of primary adrenal insufficiency, which involves the loss of mineralocorticoid as well as glucocorticoid, leading to more-profound blood pressure and electrolyte abnormalities.(63) Monitoring is also affected by localization, as p”
- Surviving Sepsis Campaign International Guidelines for the Management of Septic Shock and Sepsis-Associated Organ Dysfunction in Children (2020, background): “Interpretation should focus on the baseline serum ACTH concentration (above normal indicating primary adrenal insufficiency) and the 60-minute stimulated serum cortisol concentration (< 18 μg/dL indicating primary adrenal insufficiency) (337).”