Hypercortisolism

Excerpt

Hypercortisolism (HCM) refers to the clinical state resulting from excessive tissue exposure to cortisol and/or other related glucocorticoids. It is often but not always associated with excess serum cortisol (hypercortisolemia) and, when sustained over some time, results in the distinctive syndrome known as Cushing syndrome. Cushing syndrome (CS) is the cluster of clinical signs and symptoms resulting from long-term systemic exposure to excess cortisol and/or other glucocorticoids, from either exogenous or endogenous sources. Exogenous CS is most typically the result of the iatrogenic or surreptitious administration of glucocorticoids. Endogenous CS includes two major sub-types: adrenocorticotropic hormone (ACTH) dependent and ACTH independent. This clinical syndrome continues to be linked with Harvey W. Cushing, MD, whose original description of patients with endogenous HCM due to pituitary adenoma remains a seminal classic. The importance of his contributions to the recognition of this entity shows by the fact that pituitary based ACTH dependent HCM is still referred to worldwide as Cushing disease (CD).

The difficulty inherent in the recognition, diagnosis, and subsequent management of HCM is due to the wide range of clinical presentations that it can have. While classic advanced CS has pathognomonic clinical features which are quite obvious with characteristic laboratory findings, that form of HCM is rather uncommon. Other forms of HCM including subclinical Cushing syndrome (SCS), iatrogenic Cushing syndrome, exogenous Cushing syndrome, factitious, cyclical, intermittent, and pseudo-Cushing syndrome (aka physiologic HCM) as a group are way more common than classic CS and often more challenging to recognize, diagnose and manage.