Nicola Neary1, Lynnette Nieman. 1. Program on Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892-1109, USA.
Abstract
PURPOSE OF REVIEW: Adrenal insufficiency, first codified in 1855 by Thomas Addison, remains relevant in 2010 because of its lethal nature. RECENT FINDINGS: Reports illuminate features of adrenal insufficiency cause, diagnosis and treatment, and the role of glucocorticoids in critical illness. SUMMARY: Progress has been made in identifying human leukocyte antigen and major histocompatability complex alleles that predispose to the development of adrenal insufficiency in patients with antibodies to 21-hydroxylase, but their role in clinical care is not established. Reports of HIV-associated infections and medication-induced hypocortisolism are reminders that autoimmune adrenal destruction does not underlie all cases. The diagnosis is adequately established by the 250 microg adrenocortocotropin hormone stimulation test in most patients; the 1 microg test carries the risk of misdiagnosis of healthy individuals as adrenally insufficient. Glucocorticoids provide life-saving treatment, but long-term quality of life is impaired, perhaps because therapy is not given in a physiologic way. The current recommended total daily dose is lower than that often prescribed. Dehydroepiandrosterone replacement may be useful in pubertal girls with hypopituitarism, but not in adults. Supraphysiologic hydrocortisone doses may aid in the reversal of septic shock independent of underlying adrenal function.
PURPOSE OF REVIEW: Adrenal insufficiency, first codified in 1855 by Thomas Addison, remains relevant in 2010 because of its lethal nature. RECENT FINDINGS: Reports illuminate features of adrenal insufficiency cause, diagnosis and treatment, and the role of glucocorticoids in critical illness. SUMMARY: Progress has been made in identifying human leukocyte antigen and major histocompatability complex alleles that predispose to the development of adrenal insufficiency in patients with antibodies to 21-hydroxylase, but their role in clinical care is not established. Reports of HIV-associated infections and medication-induced hypocortisolism are reminders that autoimmune adrenal destruction does not underlie all cases. The diagnosis is adequately established by the 250 microg adrenocortocotropin hormone stimulation test in most patients; the 1 microg test carries the risk of misdiagnosis of healthy individuals as adrenally insufficient. Glucocorticoids provide life-saving treatment, but long-term quality of life is impaired, perhaps because therapy is not given in a physiologic way. The current recommended total daily dose is lower than that often prescribed. Dehydroepiandrosterone replacement may be useful in pubertal girls with hypopituitarism, but not in adults. Supraphysiologic hydrocortisone doses may aid in the reversal of septic shock independent of underlying adrenal function.
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