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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 none of the symptoms or signs are pathognomonic of the syndrome. 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 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, so 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'.)

Ectopic ACTH syndrome — This is probably the second most common cause of Cushing's syndrome, but it is often not diagnosed. About 1 percent of patients with small-cell lung cancer have ectopic ACTH syndrome; small-cell lung carcinoma causes half of all cases of the syndrome [1]. The incidence of small-cell lung carcinoma has decreased, but there are still approximately 30,000 new cases annually in the United States [2]. Therefore, one could expect approximately 300 new cases of ectopic ACTH syndrome per year. (See "Causes and pathophysiology of Cushing's syndrome", section on 'Ectopic ACTH syndrome'.)

                       

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Literature review current through: Nov 2016. | This topic last updated: Mon Jul 13 00:00:00 GMT+00:00 2015.
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