Abstract
Cyclic Cushing’s syndrome (also known as intermittent or periodic) is a disease characterized by periods of transient hypercortisolemia shifting into periods of normo- and/or hypocortisolemia. Diagnosis of cyclic Cushing’s syndrome is based on at least three periods of confirmed hypercortisolemia interspersed by two periods of normocortisolemia. Cyclic Cushing’s syndrome is one of the greatest challenges in modern endocrinology due to its diverse clinical picture, unpredictable duration and frequency of phases, and various etiologies. We discuss a diagnostic algorithm for periodic hypercortisolemia with special regard to hair cortisol analysis and desmopressin stimulation test which both seem to be helpful in finding the correct answer.
Highlights
Cyclic Cushing’s syndrome is a disease characterized by periods of transient hypercortisolemia shifting into periods of normo- and/or hypocortisolemia
Certain scenarios necessitate the use of alternative initial tests such as late-night serum cortisol or two-day low-dose dexamethasone suppression test (LDDST)
Increase of plasma adrenocorticotropic hormone (ACTH) by at least 50% in ACTH-dependent cases
Summary
Cyclic Cushing’s syndrome ( known as intermittent or periodic) is a disease characterized by periods of transient hypercortisolemia shifting into periods of normo- and/or hypocortisolemia. Certain scenarios necessitate the use of alternative initial tests such as late-night serum cortisol or two-day low-dose dexamethasone suppression test (LDDST). The latter one seems especially helpful due to improved specificity as compared with the overnight DST. If laboratory results are ambiguous, the secondline diagnostic approach may include LDDST with consecutive CRH administration [20, 21] (Figure 1) It is especially useful for distinguishing between Cushing’s syndrome and non-neoplastic hypercortisolemia (previously referred to as pseudo-Cushing’s syndrome). Differential diagnosis of cyclic Cushing’s syndrome should include: exogenous steroid use, mild autonomous hypercortisolemia (previously referred to as subclinical Cushing’s syndrome), non-neoplastic hypercortisolemia, use of xenobiotics affecting hormonal tests, glucocorticoid resistance syndrome, and factitious disorder. As fatal cases of factitious hypercortisolemia were reported [32], patients suspected of TABLE 1 | Diagnostic procedures commonly used in evaluation of hypercortisolemia
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