II. 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%)

III. Symptoms

  1. Mood changes (depression and euphoria)
  2. Easy Bruising
  3. Weakness
  4. Weight gain
  5. Amenorrhea
  6. Back pain

IV. Signs

  1. Truncal Obesity (90%)
  2. Hypertension (85% when Cushing Disease is caused by tumors, 20% when iatrogenic)
  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

V. 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)

VI. Radiology

  1. CT or MRI Cone down Sella Turcica
    1. Pituitary Adenoma
  2. CT Abdomen
    1. Adrenal adenoma

VII. Management

  1. Exogenous Cushing Syndrome (Iatrogenic Cushing Syndrome, most common cause)
    1. Stop Corticosteroids or decrease dose
    2. Change steroid dosing to every other day with drug holiday
  2. Endogenous Cushing Syndrome
    1. Surgically excise adenoma (in pituitary or adrenal)

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Ontology: Cushing Syndrome (C0010481)

Definition (CHV) a rare hormonal disease that occurs when the body produces too much of the hormone cortisol
Definition (CHV) a rare hormonal disease that occurs when the body produces too much of the hormone cortisol
Definition (MEDLINEPLUS)

Cushing's syndrome is a hormonal disorder. The cause is long-term exposure to too much cortisol, a hormone that your adrenal gland makes. Sometimes, taking synthetic hormone medicine to treat an inflammatory disease leads to Cushing's. Some kinds of tumors produce a hormone that can cause your body to make too much cortisol.

Cushing's syndrome is rare. Some symptoms are

  • Upper body obesity
  • Thin arms and legs
  • Severe fatigue and muscle weakness
  • High blood pressure
  • High blood sugar
  • Easy bruising

Lab tests can show if you have it and find the cause. Your treatment will depend on why you have too much cortisol. If it is because you have been taking synthetic hormones, a lower dose may control your symptoms. If the cause is a tumor, surgery and other therapies may be needed.

NIH: National Institute of Diabetes and Digestive and Kidney Diseases

Definition (NCI) A syndrome caused by high levels of cortisol in the blood either due to excessive production and secretion of corticosteroids secondary to pituitary or adrenocortical neoplasms, or intake of glucocorticoid drugs. Signs and symptoms include a round face, upper body obesity, fragile and thin skin, purple stretch marks in the skin, fatigue, muscle weakness, hypertension, diabetes mellitus, hypertrichosis and amenorrhea in women, impotence in men, and osteoporosis.
Definition (NCI_NCI-GLOSS) A condition in which there is too much cortisol (a hormone made by the outer layer of the adrenal gland) in the body. Cushing syndrome may be caused by taking too many steroid drugs or by certain types of tumors. Tumors that make adrenocorticotropic hormone (ACTH) cause the adrenal gland to make too much cortisol. Symptoms of Cushing syndrome include a round face, thin arms and legs, severe fatigue and muscle weakness, high blood pressure, high blood sugar, purple or pink stretch marks on the skin, and weight gain, especially in the abdomen.
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.
Concepts Disease or Syndrome (T047)
MSH D003480
ICD9 255.0
ICD10 E24 , E24.9
SnomedCT 154705004, 190505004, 47270006
English Cushing Syndrome, Cushing's Syndrome, Syndrome, Cushing's, CUSHING'S SYNDROME, Cushing's syndrome, unspecified, Cushing syndrome, Cushing's basophilism, pituitary basophilism, Syndrome, Cushing, Cushing's syndrome (diagnosis), Cushings syndrome, Syndrome Cushing's, Cushing Syndrome [Disease/Finding], cushing's syndrome, cushing syndrome, Cushing's syndrome (disorder), Cushing's syndrome NOS (disorder), Cushing's syndrome, Cushing's syndrome III, Itsenko disease, Itsenko-Cushing syndrome, Suprarenogenic syndrome, Cushing; syndrome or disease, Hypercortisolism, Cushings Syndrome, Cushing's syndrome NOS, cushings syndrome
Portuguese SINDROME DE CUSHING, Síndrome de Cushing
Spanish SINDROME DE CUSHING, síndrome de Cushing, SAI (trastorno), síndrome de Cushing, SAI, síndrome de Cushing, síndrome de Itsenko - Cushing, Síndrome de Cushing
Dutch syndroom van Cushing, Cushing; syndroom of ziekte, Syndroom van Cushing, niet gespecificeerd, Cushing-syndroom, Syndroom van Cushing, Syndroom, Cushing-
German Syndrom, Cushing, CUSHING SYNDROM, Cushing-Syndrom, nicht naeher bezeichnet, Cushing Syndrom, Cushing-Syndrom
Swedish Cushings syndrom
Japanese クッシングショウコウグン, Cushing症候群, クッシング症候群, 下垂体好塩基細胞腺腫, 脳下垂体好塩基細胞腺腫
Czech Cushingův syndrom, hyperkortizolismus
Finnish Cushingin oireyhtymä
Russian ITSENKO-KUSHINGA BOLEZN', ITSENKO-KUSHINGA SINDROM, KUSHINGA SINDROM, ИЦЕНКО-КУШИНГА БОЛЕЗНЬ, ИЦЕНКО-КУШИНГА СИНДРОМ, КУШИНГА СИНДРОМ
French SYNDROME DE CUSHING, Syndrome de Cushing, Hypercortisolisme
Italian Ipercortisolismo, Sindrome di Cushing
Korean 쿠싱 증후군, 상세불명의 쿠싱 증후군
Croatian CUSHINGOV SINDROM
Polish Zespół Cushinga
Hungarian Cushing syndroma, Cushing-syndroma, Cushing-tünetegyüttes
Norwegian Cushings syndrom, Hyperkortisolisme