Endocrinology Book

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Cushing's Disease

Aka: Cushing's Disease, 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 332:791-803

Cushing Syndrome (C0010481)

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 (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)

Cushings syndrome is caused by 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 secrete 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

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 a tumor caused it, surgery and other therapies may be needed. Most of the time, Cushing's syndrome can be cured.

NIH: National Institute of Neurological Disorders and Stroke
Definition (NCI) 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 (NCI) A condition in which there is too much cortisol (a hormone made by the outer layer of the adrenal gland) in the body. In Cushing disease, this happens when an adenoma (benign tumor) in the pituitary gland makes too much adrenocorticotropic hormone (ACTH). This causes the adrenal gland to make too much cortisol. Symptoms include a round face, thin arms and legs, severe fatigue and muscle weakness, high blood pressure and 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, CUSHINGS SYNDROME, CUSHING SYNDROME, CUSHING'S SYNDROME, SYNDROME CUSHING'S, Cushing's syndrome NOS, Cushing's syndrome, unspecified, Cushing syndrome, Cushing's basophilism, pituitary basophilism, Syndrome, Cushing, Cushing's syndrome (diagnosis), Cushing's syndrome (disorder), Cushings syndrome, Syndrome Cushing's, CUSHING, Cushing's syndrome NOS (disorder), Cushing Syndrome [Disease/Finding], cushing's syndrome, cushing syndrome, Cushing disease, Cushing's syndrome, Cushing's syndrome III, Itsenko disease, Itsenko-Cushing syndrome, Suprarenogenic syndrome, Hypercortisolism, Cushings Syndrome, cushings syndrome
Portuguese SINDROME DE CUSHING, Síndrome de Cushing
Spanish SINDROME DE CUSHING, Cushing's syndrome, síndrome de Cushing, SAI (trastorno), síndrome de Cushing, SAI, síndrome de Cushing, síndrome de Itsenko - Cushing, Sindrome de Cushing, Síndrome de Cushing
Dutch syndroom van Cushing, 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 ｸｯｼﾝｸﾞｼｮｳｺｳｸﾞﾝ, 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
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
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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