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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Excess cortisol in the circulation

  • Central obesity

  • Hypertension

  • Hyperlipidemia

  • Hyperglycemia

GENERAL CONSIDERATIONS

  • Cushing’s syndrome can be caused by an adrenocorticotropic hormone (ACTH)-producing pituitary tumor (Cushing’s disease); ACTH production by other tumors, such as small-cell carcinoma of the bronchus; glucocorticoid-secreting adrenal tumors; or exogenous steroid use

  • Because of associated central obesity, hypertension, hyperglycemia, and hyperlipidemia, affected patients are at risk for coronary artery disease

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Fatigue, dyspnea, and chest pain

PHYSICAL EXAM FINDINGS

  • Central obesity, dorsal hump, plethoric facies, striae

  • Signs of cardiac dysfunction

DIFFERENTIAL DIAGNOSIS

  • Pseudo-Cushing’s syndrome: patients appear cushingoid but have normal screening laboratory tests

  • Other causes of obesity, hypertension, hyperlipidemia, and hyperglycemia

DIAGNOSTIC EVALUATION

LABORATORY TESTS

Screening tests

  • Twenty-four-hour urine for free cortisol

  • Overnight dexamethasone suppression test

Confirmatory tests

  • Two-day dexamethasone suppression test

  • Corticotropin-releasing factor test

Determine cause of abnormal ACTH

  • ACTH level

    • – Elevated ACTH indicates pituitary tumor or ectopic production

    • – Low ACTH indicates adrenal disease

  • High-dose dexamethasone suppression test and corticotropin-releasing hormone stimulation test will differentiate pituitary from ectopic sources

  • Inferior petrosal sinus sampling when hormone tests unclear but a pituitary tumor is seen on imaging or an ectopic source is suspected

ELECTROCARDIOGRAPHY

  • Signs of myocardial ischemia/infarction may be present

IMAGING STUDIES

  • CT scan usually identifies the tumor(s)

TREATMENT

CARDIOLOGY REFERRAL

  • Suspected cardiac disease

HOSPITALIZATION CRITERIA

  • Acute coronary syndrome

  • Planned surgery

MEDICATIONS

  • If surgery is not feasible, adrenolytic (eg, mitotane) or adrenocortical-blocking pharmacologic agents (eg, glutethimide, metyrapone, or ketoconazole) can be used

SURGERY

  • Surgical removal of the pituitary, adrenal, or ectopic tumor is usually definitive

MONITORING

  • ECG monitoring in hospital as appropriate

DIET AND ACTIVITY

  • Low-fat diet

  • Restricted activity if heart disease is present

ONGOING MANAGEMENT

HOSPITAL DISCHARGE CRITERIA

  • Resolution of problem

  • Successful surgery

FOLLOW-UP

  • Depends on condition

COMPLICATIONS

  • Myocardial infarction

  • Heart failure

  • Cardiovascular death

PROGNOSIS

  • Good with recognition and treatment. The longer the condition persists, the more likely coronary artery disease will occur

RESOURCES

PRACTICE GUIDELINES

  • Diagnosis requires 3 steps:

    • – Screening for ...

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