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Cushing's DiseaseAka: Cushing's Syndrome

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  1. Etiologies
    1. Iatrogenic
      1. Steroid therapy (most common cause)
    2. Central Cause
      1. Pituitary adenoma
    3. Adrenal Cause
      1. Adrenal Adenoma
      2. Adrenal Hyperplasia
      3. Adrenal Malignancy (15%)
    4. Ectopic Source
      1. Malignancy (Small Cell Carcinoma of the lung: 15%)
  2. Symptoms
    1. Mood changes (depression and euphoria)
    2. Easy bruising
    3. Weakness
    4. Weight gain
    5. Amenorrhea
    6. Back pain
  3. Signs
    1. Truncal Obesity (90%)
    2. Hypertension (85%)
    3. Glucose Intolerance (80%)
    4. Hirsutism (70%)
    5. Wide, purple abdominal and thigh striae (65%)
    6. Osteoporosis (55%)
    7. Moon facies
    8. Buffalo hump (Thoracic kyphosis)
    9. Myopathy
    10. Plethoric face
    11. Supraclavicular fat pad development
    12. Hypertrichosis
    13. Peripheral Edema
    14. Hypertension
  4. Labs
    1. Screening Test
      1. 24-hour Urinary free cortisol level (preferred)
        1. Urine 17-Ketosteroid excretion
        2. Urine 17-Hydroxysteroid excretion
      2. Serum Cortisol
      3. Low dose Dexamethasone Suppression Test
        1. Dexamethasone 1 mg at 11pm
        2. Plasma Cortisol in following 8 AM
      4. Night-time Salivary cortisol testing
    2. Distinguish between pituitary, adrenal or ectopic cause
      1. Plasma ACTH
      2. High dose Dexamethasone Suppression Test (8 mg)
  5. Radiology
    1. CT or MRI Cone down Sella Turcica
      1. Pituitary adenoma
    2. CT abdomen
      1. Adrenal tumor
  6. Management
    1. Exogenous Cushing's
      1. Stop steroids or decrease dose
      2. Change steroid dosing to qod with drug holiday
    2. Endogenous Cushing's
      1. Surgically excise adenoma (in pituitary or adrenal)
  7. Resources
    1. Addison and Cushing International Federation (ACIF)
      1. http://www.nvacp.nl/page.php?main=5
  8. References
    1. Orth (1995) N Engl J Med 332:791

Cushing Syndrome (C0010481)

Definition (MSH)A condition caused by prolonged exposure to excess levels of cortisol (HYDROCORTISONE) or other GLUCOCORTICOIDS from endogenous or exogenous sources. It is characterized by upper body OBESITY; OSTEOPOROSIS; HYPERTENSION; DIABETES MELLITUS; HIRSUTISM; AMENORRHEA; and excess body fluid. Endogenous Cushing syndrome or spontaneous hypercortisolism is divided into two groups, those due to an excess of ADRENOCORTICOTROPIN and those that are ACTH-independent.
Definition (CSP)condition, more commonly seen in females, due to hyperadrenocorticism resulting from neoplasms of the adrenal cortex or the anterior lobe of the pituitary, or to prolonged excessive intake of glucocorticoids for therapeutic purposes; symptoms and signs may include rapidly developing adiposity of the face, neck, and trunk, kyphosis caused by osteoporosis of the spine, hypertension, diabetes mellitus, amenorrhea, hypertrichosis in females, impotence in males, dusky complexion with purple markings, polycythemia, pain in the abdomen and back, and muscular wasting and weakness.
ConceptsDisease or Syndrome (T047)
ICD9255.0, 255.0
MSHD003480
EnglishCushing Syndrome, Cushing's basophilism, Cushing's Syndrome, Cushing's syndrome III, CUSHINGS SYNDROME, Hypercortisolism, Itsenko disease, Itsenko-Cushing syndrome, pituitary basophilism, Suprarenogenic syndrome, SYNDROME CUSHING'S
Spanishhiperfuncion corticoadrenal, hiperfuncion corticosuprarrenal, sindrome de Cushing, sindrome de Itsenko - Cushing
Parent ConceptsAdrenal cortical hypofunction (C0405580), Adrenal Gland Diseases (C0001621), Syndrome (C0039082), Endocrine System Disorders, General, Polyglandular and NEC (C0549525), ADRENAL DYSFUNCTION (C0549609), Adrenal Gland Hyperfunction (C0001622), Endocrine Syndrome (C1333387), Vascular Hemostatic Disorders (C0600502), Duplicate concept (C1274013)
SourcesAOD, COSTAR, CSP, CST, DXP, ICD9CM, LCH, MEDLINEPLUS, MSH, MTH, MTHICD9, NCI, NDFRT, OMIM, PDQ, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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