Cushing's Syndrome for the Nurse Practitioner Licensing Exam
- Pathophysiology
- Excess Cortisol Production: Cushing’s syndrome is caused by prolonged exposure to elevated levels of cortisol, leading to systemic effects on metabolism, immune function, and cardiovascular health.
- ACTH-Dependent vs. ACTH-Independent:
- ACTH-Dependent: Results from excessive production of adrenocorticotropic hormone (ACTH), which stimulates the adrenal glands to produce cortisol.
- Cushing's Disease: A pituitary adenoma secreting ACTH is the most common endogenous cause.
- Ectopic ACTH Production: Non-pituitary tumors (e.g., small-cell lung carcinoma) secrete ACTH.
- ACTH-Independent: Autonomous cortisol production by the adrenal glands.
- Adrenal Adenoma/Carcinoma: Adrenal tumors independently secrete cortisol.
- Exogenous Glucocorticoid Use: The most common cause overall, due to long-term steroid therapy.
- Clinical Features
- General Appearance:
- Central Obesity: Fat deposition in the trunk with relatively thin extremities.
- Moon Facies: A rounded, full face.
- Buffalo Hump: Fat accumulation in the upper back.
- Skin and Hair:
- Purple Striae: Stretch marks, usually on the abdomen and thighs.
- Thin Skin and Easy Bruising: Fragile skin with poor wound healing.
- Hirsutism: Excess hair growth, especially in women.
- Metabolic and Musculoskeletal:
- Hyperglycemia: Cortisol increases gluconeogenesis and insulin resistance.
- Hypertension: Elevated blood pressure due to sodium retention.
- Osteoporosis: Increased risk of fractures due to decreased bone formation.
- Proximal Muscle Weakness: Weakness, especially in the thighs and upper arms.
- Diagnosis
- 24-Hour Urine Free Cortisol: Measures cortisol levels over 24 hours. Elevated levels confirm hypercortisolism.
- Late-Night Salivary Cortisol: Cortisol should be low at night; elevated levels suggest Cushing's syndrome.
- Low-Dose Dexamethasone Suppression Test: Failure to suppress cortisol after dexamethasone indicates Cushing’s syndrome.
- ACTH Levels: Elevated in ACTH-dependent Cushing’s, low in ACTH-independent causes.
- Treatment
- Surgery:
- Pituitary Adenoma: Transsphenoidal surgery is the treatment of choice for Cushing’s disease.
- Adrenal Tumors: Adrenalectomy is indicated for adrenal adenomas or carcinomas.
- Ectopic ACTH-Producing Tumors: Surgical resection, if feasible.
- Medical Therapy: Steroidogenesis inhibitors (e.g., ketoconazole) or glucocorticoid receptor antagonists (e.g., mifepristone) for non-surgical candidates.
Key Points
- Pathophysiology: Cushing's syndrome results from excess cortisol due to pituitary adenomas (Cushing’s disease), adrenal tumors, ectopic ACTH production, or exogenous steroids.
- Clinical Features: Central obesity, moon facies, purple striae, muscle weakness, hyperglycemia, and hypertension are key findings.
- Diagnosis: Includes 24-hour urine free cortisol, late-night salivary cortisol, and low-dose dexamethasone suppression test.
- Treatment: Surgery is the main treatment for pituitary or adrenal tumors; medical management is used when surgery is not an option.