Primary Generalized Glucocorticoid Resistance Treatment & Management

Updated: Sep 28, 2016
  • Author: ; Chief Editor: Robert P Hoffman, MD  more...
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Treatment

Medical Care

In Chrousos syndrome, the goal of treatment is to suppress the excess secretion of corticotropin, thereby suppressing the increased production of adrenal steroids with mineralocorticoid and androgenic activity. Treatment involves administration of high doses of mineralocorticoid-sparing synthetic glucocorticoids, such as dexamethasone (1-3 mg given once daily at night), which activate the mutant and/or wild-type hGRα and suppress the endogenous secretion of corticotropin in affected subjects. [20, 21, 22, 45] It is important to achieve adequate suppression of the HPA axis to prevent the development of a corticotropin-secreting adenoma in the pituitary gland and an adrenocortical adenoma in the adrenal gland. [29] Long-term dexamethasone treatment should be carefully titrated according to the clinical manifestations and biochemical profile of the affected subjects. [20, 21, 22, 45]

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Long-Term Monitoring

Regular follow-up appointments in which body weight and blood pressure are monitored are necessary. Clinical and biochemical indices of glucocorticoid action should also be monitored.

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