disease | Cortisol Excess Syndrome |
alias | Cushing's Syndrom |
Also known as Cushing's syndrome, it is a disease caused by the excessive secretion of glucocorticoids from the adrenal cortex. Depending on the disease cause, it is divided into Cushing's disease (excessive secretion of ACTH by the pituitary gland), Cushing's syndrome (excessive secretion of glucocorticoids by the adrenal glands), and ectopic ACTH syndrome (production of ACTH by tumors outside the pituitary gland).
bubble_chart Diagnosis
1. History and Symptoms:
Most cases start with obesity, presenting with a moon face and central obesity. There are purple striae on the lower abdomen and inner thighs. The facial appearance is plethoric, with menstrual disorders or amenorrhea in women and decreased libido in men. Some cases of ectopic ACTH syndrome with grade III Cushing's disease may have skin pigmentation.
2. Physical Examination Findings:
Elevated blood pressure, thin skin, acne, purple striae on the lower abdomen and limbs, hirsutism or pigmentation, central obesity, moon face, etc.
3. Auxiliary Examinations:
(1) Pituitary Cushing's disease; increased urinary 17-OH, urinary 17-KS grade II, positive high-dose dexamethasone suppression test, slightly elevated plasma ACTH in the morning with no significant decrease at night, CRH, ACTH stimulation test, and metyrapone test above normal, severe cases may have hypokalemic alkalosis.
(2) Sella turcica/adrenal CT/MRI, adenoma/bilateral hyperplasia may be seen. In adrenal cortical adenoma, urinary 17-OH grade II is increased, urinary 17-KS may be normal or increased, high-dose dexamethasone suppression test is negative, plasma ACTH is decreased, CRH, ACTH stimulation test, and metyrapone test are mostly non-reactive, adrenal CT scan can show the tumor.
bubble_chart Treatment Measures
1. Pituitary Cushing's Disease: The first choice is transsphenoidal surgery to remove pituitary microadenomas/macroadenomas. For those without detectable adenomas or who are not suitable for surgery, subtotal adrenalectomy can be performed, followed by pituitary radiotherapy. Without radiotherapy, the risk of developing Nelson's syndrome is higher. Bromocriptine, cyproheptadine, etc., can be used to treat this disease and Nelson's syndrome.
2. Patients with Adrenal Tumors: Surgical removal can achieve a cure, followed by cortisol replacement therapy, gradually reducing the dose until discontinuation. For ectopic ACTH syndrome, treatment options include surgery, radiation, chemotherapy, and medications such as metyrapone, depending on the specific condition. It is important to strengthen the follow-up and review of treated patients during diagnosis and treatment.
It should be differentiated from patients with simple obesity, type II diabetes with obesity, iatrogenic Cushing's syndrome, and depression.