Cushing's syndrome resident survival guide

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

This section provides a short and straight to the point overview of the disease or symptom. The first sentence of the overview must contain the name of the disease.

Causes

Life Threatening Causes

Life-threatening causes include conditions that may result in death or permanent disability within 24 hours if left untreated.

  • Does not include any known cause.

Common Causes

Diagnosis

Shown below is an algorithm summarizing the diagnosis of Cushing's syndrome according to the Endocrine Society clinical guidelines.

 
 
 
Symptoms suggestive of Cushing’s syndrome:
  • Osteoporosis unusual for the age
  • Refractory hypertension
  • Facial edema resulting in Moon-like face
  • Central obesity
  • Proximal muscle weakness
  • Abdominal striae
  • Easy bruising
  • Menstrual abnormalities: oligomenorrhea
  • Mood disorders, depression
  • Hirtuism
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Take a detailed history of the patient including medication history. Rule out the use of any exogenous topical, oral, parenteral, or inhaled glucocorticoid and synthetic progesterone.
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Perform any two of the following three laboratory investigations:
  • Measure late-night levels of salivary cortisol (twice)
  • Measure 24 hours urinary free cortisol (UFC) excretion (twice)
  • Perform low-dose (1mg) overnight dexamethasone suppression test (DST)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Two of the following abnormal results:
  • Elevated levels of late-night salivary cortisol
  • 24 hours UFC threefold greater than the upper reference range.
  • Early morning (8 am) sample having serum cortisol 1.8 mcg/dL or higher after overnight DST
 
 
Any one abnormal result
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
High suspicion for Cushing's syndrome
 
 
Low suspicion for Cushing's syndrome
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Measure late night plasma ACTH levels
 
 
Refer to endocrinologist
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Low value plasma ACTH <5 pg/mL
 
Indeterminate values of plasma ACTH between 5 to 20 pg/mL
 
High value plasma ACTH >20 pg/mL
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
CRH or desmopressin stimulation test
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
No ACTH response
 
+ ACTH response
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
ACTH independent Cushing's syndrome
 
 
 
 
 
 
 
 
 
ACTH dependent Cushing's syndrome
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
CT scan/ MRI of adrenal glands to look for adrenal tumors or hyperplasia.
 
 
 
 
 
 
 
Perform both tests:
  • CRH or desmopressin stimulation test
  • High dose (8mg) overnight DST
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Adequate suppresion of early morning serum cortisol (with levels less than 5 mcg/dL) and stimulation with CRH
 
 
 
Negative or equivocal response
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
MRI of the pituitary
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Tumor >6mm
 
 
Tumor <6mm or no mass lesion
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Cushing's disease
 
 
 
 
 
 
 
 
Perform Inferior petrosal sinus sampling.
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Central step-up
 
 
 
No Central step-up
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Ectopic ACTH production

Treatment

Shown below is an algorithm summarizing the treatment of Cushing's syndrome according the the Endocrine Society clinical practice guidelines.

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Do's

  • The content in this section is in bullet points.

Don'ts

  • The content in this section is in bullet points.

References


Template:WikiDoc Sources

 
 
 
 
 
 
 
The treatment depends upon the underlying etiology
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Cushing's disease
 
 
 
Paraneoplastic Cushing's syndrome
 
 
 
 
ACTH-independent Cushing's syndrome
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Transsphenoidal surgery: Treatment of choice in patients with pituitary adenoma with distinct margins.
 
Resectable tumor
 
 
Nonresectable tumor
 
Adrenal adenomas
 
 
Bilateral adrenal hyperplasia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Pituitary irradiation carried out in patients with:
  • Failure to resect tumor completely by surgery.
  • The location of the tumor cannot be determined.
  • The patient wishes to conceive in the future.
 
 
Bilateral adrenalectomy is carried out in patients with severe refractory hypercortisolism.
 
Resection of the primary tumor
 
Unilateral adrenalectomy
 
Bilateral adrenalectomy