Cushing's Disease And Syndrome Disease

BASICS

DESCRIPTION
Clinical abnormalities associated with chronic exposure to excessive amounts of cortisol (the major adrenocorticoid). The most frequent cause is prolonged use of exogenous glucocorticoids.
  • System(s) affected: Endocrine/Metabolic, Musculoskeletal, Skin/Exocrine, Cardiovascular
  • Genetics:
    • Multiple endocrine neoplasia type I
    • Carney complex
  • Incidence/Prevalence in USA: Uncommon
  • Predominant age: All ages
  • Predominant sex: Females > Males
SIGNS AND SYMPTOMS
  • Moon face (facial adiposity)
  • Increased adipose tissue in neck and trunk
  • Central weight gain
  • Emotional lability
  • Hypertension
  • Osteoporosis
  • Purple striae on the skin
  • Diabetes or glucose intolerance with fasting hyperglycemia and/or glycosuria
  • Muscle weakness due to loss of muscle mass from increased catabolism
  • Skeletal growth retardation in children
  • Easy bruising
  • Hirsutism
CAUSES
  • Exogenous glucocorticoids and/or ACTH
  • Endogenous ACTH-dependent hypercortisolism
    • ACTH-secreting pituitary tumor
    • Ectopic ACTH production (e.g., small-cell carcinoma of lung, bronchial carcinoid)
  • Endogenous ACTH-independent hypercortisolism
    • Adrenal adenoma
    • Adrenal carcinoma
    • Macro/micro nodular hyperplasia
RISK FACTORS
  • Any medical problem requiring prolonged use of corticosteroids
  • Pituitary tumor
  • Adrenal mass
  • Neuroendocrine tumor (e.g., bronchial carcinoid)

DIAGNOSIS

LABORATORY
  • 24 hour urinary cortisol
  • Plasma cortisol (am and pm)
  • Plasma ACTH concentration
  • Glycosuria (possible)
  • Neutrophilia
  • Lymphopenia
  • Hyperglycemia
  • Hyperlipidemia
  • Hypokalemia
  • Dynamic endocrine testing (e.g., dexamethasone suppression test)

Drugs that may alter lab results: Refer to lab test or drug reference
Disorders that may alter lab results: Refer to lab test reference

PATHOLOGICAL FINDINGS
  • Hyalinization of basophilic cells (anterior pituitary) - Crooke's cell changes
  • Muscular atrophy
  • Nephrosclerosis
SPECIAL TESTS
If ACTH-dependent, inferior petrosal sinus sampling for ACTH
IMAGING
  • Chest films
  • X-rays of the lumbar spine - osteoporosis is common
  • If pituitary tumor suspected - pituitary MRI scan
  • If adrenal disease suspected - abdominal CT scan
  • If ectopic ACTH-secretion suspected - chest CT scan
DIAGNOSTIC PROCEDURES
Not all tests indicated for every case. Choice of diagnostic procedure dependent on circumstances and judgment.

TREATMENT

APPROPRIATE HEALTH CARE

Inpatient for surgical procedures

GENERAL MEASURES
  • Depends on etiology. Surgery is the treatment of choice; persistent disease may require - radiation, drug therapy, or surgery/li>
  • Medical treatment with adrenocortical inhibitors
    • Has not been too successful
    • Should be used when other methods fail
    • In consultation with a clinician having experience in their use
SURGICAL MEASURES
  • Primary hypersecretion of ACTH
    • Transsphenoidal microsurgery. Bilateral adrenalectomy as an adjunct for patients not cured.
  • Adrenocortical tumors
    • Surgical removal when possible
    • If adrenocortical carcinoma, prognosis is poor
  • Ectopic ACTH production
    • Removal of the neoplastic tissue
    • Metastatic spread makes surgical cure unlikely/impossible
    • Bilateral adrenalectomy
ACTIVITY

Determined by patient's symptoms and form of treatment used

DIET
  • Potassium supplements
  • High protein diet
PATIENT EDUCATION
  • National Adrenal Disease Foundation (NADF), 505 Northern Blvd, Great Neck, NY 11021; 516-407-4992; e-mail: nadf@aol.com
  • Instructions on drug therapy, diet, activity
  • Early treatment of infections
  • Monitor weight daily
  • Emotional lability prevention

FOLLOW UP

PREVENTION/AVOIDANCE
  • Avoid excessive corticosteroid treatment when possible
  • Comprehensive teaching to help patient cope with lifelong treatment (if needed)
POSSIBLE COMPLICATIONS
  • Osteoporosis
  • Increased susceptibility to infections
  • Hirsutism
  • Metastases of malignant tumors
EXPECTED COURSE AND PROGNOSIS
  • Usual course - chronic with cyclic exacerbations and rare remissions
  • Guardedly favorable prognosis with surgery

MISCELLANEOUS

ASSOCIATED CONDITIONS

Tumors of the pituitary; multiple endocrine neoplasia Type I, Carney complex

AGE-RELATED FACTORS

Pediatric: Rare in infancy and childhood. Most cases (under age 8) are due to malignant adrenal tumors.
Geriatric: N/A
Others: N/A

PREGNANCY

Can cause exacerbation of Cushing's disease

OTHER NOTES

Pituitary ACTH excess due to a pituitary tumor causes what is called Cushing's disease

ABBREVIATIONS

CRH = corticotropin releasing hormone

Clinical Investigations

ROLE OF HOMOEOPATHY

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