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Epidemiology and clinical manifestations of Cushing's syndrome

Author
Lynnette K Nieman, MD
Section Editor
André Lacroix, MD
Deputy Editor
Kathryn A Martin, MD

INTRODUCTION

The symptoms and signs of Cushing's syndrome result directly from chronic exposure to excess glucocorticoid. Establishing the diagnosis is often difficult because few of the symptoms or signs are pathognomonic of the syndrome in isolation. There is a large spectrum of manifestations from subclinical to overt syndrome, depending on duration and intensity of excess steroid production. Furthermore, some of them (such as obesity, hypertension, and glucose intolerance) are common in individuals who do not have adrenal hyperfunction. An important clinical clue to the presence of glucocorticoid excess is the simultaneous development and increasing severity of several of these symptoms.

The major manifestations of Cushing's syndrome will be reviewed here. The diagnosis and treatment of this disorder and Cushing's syndrome during pregnancy are discussed separately. (See "Establishing the diagnosis of Cushing's syndrome" and "Establishing the cause of Cushing's syndrome" and "Overview of the treatment of Cushing's syndrome" and "Cushing's syndrome in pregnancy".)

EPIDEMIOLOGY

Estimates of the incidence of Cushing's syndrome are imprecise and likely underestimate the incidence of iatrogenic Cushing's syndrome, undiagnosed mild hypercortisolism, and the ectopic corticotropin (ACTH) syndrome.

Iatrogenic Cushing's syndrome More than 10 million Americans receive pharmacologic doses of glucocorticoids each year. Therefore, iatrogenic Cushing's syndrome must be more common than any other cause but is seldom reported. (See "Major side effects of systemic glucocorticoids", section on 'Cushingoid appearance and weight gain'.)

Cushing's disease Pituitary ACTH-dependent Cushing's syndrome is five to six times more common than Cushing's syndrome caused by benign and malignant adrenal tumors combined [1]. Thus, the incidence of Cushing's disease may be 5 to 25 per million per year. However, the reported incidence was much lower (1.2 to 2.4 per million per year) in one population-based study [2,3]. In a United States study, the incidence was higher than previously reported in European studies as it was found to affect 6.2 to 7.6 per million person-years [4]. (See "Causes and pathophysiology of Cushing's syndrome", section on 'Cushing's disease'.)

                          

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Literature review current through: Jan 2017. | This topic last updated: Wed Jan 11 00:00:00 GMT+00:00 2017.
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