Primary adrenal failure has a prevalence of around 1/20,000. In the past, the primary cause was tuberculosis, but in the developed world most cases are now due to autoimmune adrenal failure, diagnosed in its early stages by the presence of antibodies to the enzyme 21-hydroxylase (although these decrease over time). This may be associated with other auto-immune diseases such as Hashimoto’s thyroiditis, premature ovarian failure, hypoparathyroidism, pernicious anaemia and type 1 diabetes mellitus. There is some evidence that the actual tissue destruction is T-cell mediated. Other causes included metastatic adrenal infiltration, bilateral adrenal haemorrhage, lupus anti-coagulant, histoplasmosis and various genetic disorders (familial glucocorticoid deficiency, primary adrenal hypoplasia, adrenoleukodystrophy, etc.). The symptoms are often very nonspecific, including malaise, nausea and vomiting, but on examination the hypovolaemia is associated with skin pigmentation due to activation of melanotrophs by the high circulating of ACTH. Diagnosis is made by the association of low serum cortisol with high levels of ACTH, but more rapidly by the subnormal response of serum cortisol to Synacthen (ACTH 1-24). Plasma renin is elevated and aldosterone suppressed. Secondary adrenal failure is due to hypothalamic or pituitary dysfunction, and the symptoms and signs are often milder. Cortisol is low, as is plasma ACTH, but renin and aldosterone levels are normal. Treatment is with hydrocortisone, usually best in divided doses (typically 10, 5 and 5 mg daily), plus fludrocortisone once-daily in the case of primary adrenal failure. There are slow-release forms of hydrocortisone becoming available. Doses of hydrocortisone need to be increased during acute stress and an emergency pack of hydrocortisone should be available. Care must be taken to avoid under-replacement and consequent on-going fatigue, or over-replacement and the metabolic syndrome. Some patients may additionally benefit from DHEA.
Hypoadrenalism: Primary and secondary adrenal failure / Minnetti, Marianna; Grossman, Ashley B.. - ELETTRONICO. - (2015), pp. 91-114.
Hypoadrenalism: Primary and secondary adrenal failure
Minnetti, Marianna;
2015
Abstract
Primary adrenal failure has a prevalence of around 1/20,000. In the past, the primary cause was tuberculosis, but in the developed world most cases are now due to autoimmune adrenal failure, diagnosed in its early stages by the presence of antibodies to the enzyme 21-hydroxylase (although these decrease over time). This may be associated with other auto-immune diseases such as Hashimoto’s thyroiditis, premature ovarian failure, hypoparathyroidism, pernicious anaemia and type 1 diabetes mellitus. There is some evidence that the actual tissue destruction is T-cell mediated. Other causes included metastatic adrenal infiltration, bilateral adrenal haemorrhage, lupus anti-coagulant, histoplasmosis and various genetic disorders (familial glucocorticoid deficiency, primary adrenal hypoplasia, adrenoleukodystrophy, etc.). The symptoms are often very nonspecific, including malaise, nausea and vomiting, but on examination the hypovolaemia is associated with skin pigmentation due to activation of melanotrophs by the high circulating of ACTH. Diagnosis is made by the association of low serum cortisol with high levels of ACTH, but more rapidly by the subnormal response of serum cortisol to Synacthen (ACTH 1-24). Plasma renin is elevated and aldosterone suppressed. Secondary adrenal failure is due to hypothalamic or pituitary dysfunction, and the symptoms and signs are often milder. Cortisol is low, as is plasma ACTH, but renin and aldosterone levels are normal. Treatment is with hydrocortisone, usually best in divided doses (typically 10, 5 and 5 mg daily), plus fludrocortisone once-daily in the case of primary adrenal failure. There are slow-release forms of hydrocortisone becoming available. Doses of hydrocortisone need to be increased during acute stress and an emergency pack of hydrocortisone should be available. Care must be taken to avoid under-replacement and consequent on-going fatigue, or over-replacement and the metabolic syndrome. Some patients may additionally benefit from DHEA.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
