Kikuchi disease

Disease Description (Causes and Symptoms)

Cushing syndrome is a condition that occurs when the body is excessively exposed to glucocorticoids (mainly cortisol), and its causes can be broadly divided as follows.

• Exogenous: Long-term or high-dose use of oral/injected steroids (adrenocortical hormones)
• Endogenous
  • ACTH-secreting pituitary tumor (Cushing disease, ACTH-dependent)
  • Adrenal tumor (excessive cortisol production in the adrenal cortex, ACTH-independent)
  • Other causes (inappropriate ACTH secretion from lung cancer and other conditions — ectopic ACTH production) Pathophysiologically, excessive cortisol causes protein breakdown, fat redistribution, insulin resistance, increased bone resorption, immunosuppression, and other effects.

Major symptoms and findings:

• Moon face, fat accumulation in the back of the neck and shoulders (buffalo hump), and central (abdominal) obesity
• Muscle weakness (particularly proximal muscle weakness) and thin, slender limbs
• Skin atrophy, easy bruising, purple striae (vertical lines on the abdomen), acne, and hirsutism
• Hypertension, hyperglycemia (diabetes), and lipid abnormalities
• Increased risk of osteoporosis and fractures
• Mood changes (depression and anxiety) and sexual dysfunction

Treatment Methods and Precautions

Treatment varies depending on the cause, and the goals are to normalize the hypercortisol state and manage complications.

Main treatment methods

• Exogenous (steroid-induced): If medically necessary, reduce the dose as much as possible; when discontinuing, taper gradually (sudden discontinuation may cause adrenal insufficiency). Steroid discontinuation or dose reduction should be managed with the treating physician.
• Pituitary (Cushing disease): Transsphenoidal surgery is the first-line treatment. If surgery is incomplete or the disease recurs, radiation therapy or drug treatment (e.g., ketoconazole, metyrapone, osilodrostat, etc.) may be considered.
• Adrenal tumor: Surgical removal (unilateral resection, mostly laparoscopic). Temporary or long-term steroid replacement therapy may be required after surgery.
• Ectopic ACTH-secreting tumor: Treatment of the primary tumor (surgery, chemotherapy, etc.) takes priority; drug treatment may be used to control acute hypercortisolism.
• Drug therapy (medical control): Cortisol synthesis inhibitors (metyrapone, ketoconazole and levoketoconazole), mitotane (destruction of adrenal cells), cortisol secretion-inhibiting treatment (osilodrostat), and others. The indications and adverse effects differ for each medication.

Lifestyle management and complication prevention

• Management of blood pressure, blood glucose, and lipids: Dietary control, exercise, and medication when necessary
• Osteoporosis prevention: Calcium and vitamin D supplementation, weight-bearing exercise, bone density testing, and consideration of osteoporosis medications
• Infection prevention: Take care to prevent infections because immune function is reduced (including consultation about vaccination)
• Wound healing and skin care: Take care with wounds because the skin becomes thin
• Regular monitoring for cardiovascular and endocrine complications

Precautions and complications

• If untreated, the risk of hypertension, diabetes, lipid abnormalities, cardiovascular disease, osteoporosis, and infections may increase, and mental and sexual dysfunction may persist.
• After surgery or during medication tapering, there is a risk of adrenal insufficiency (low cortisol); pay attention to symptoms such as fatigue, dizziness, and low blood pressure.
• Sudden discontinuation of steroids is dangerous, so taper gradually according to medical instructions.

Outpatient Visit Checklist (Including Preparation Items and Example Questions)

Recommended items to bring

• A list of all medications and supplements currently being taken (including doses and administration times), especially whether steroid preparations are being used
• Previous test results (blood tests, 24-hour urine cortisol, hormone tests, CT/MRI images, etc.)
• A record of when symptoms began and how they have progressed (including weight changes, blood pressure changes, history of fractures, history of infections, etc.)
• Recent weight, blood pressure, and blood glucose measurements (self-monitoring records), if available, either printed or recorded in a log
• Major medical history (surgeries and history of cancer) and family history

Information that is helpful to report during an outpatient visit

• Steroids currently being taken (formulation, dose, and duration of administration) and history of steroid-related injections
• Factors that worsen or improve symptoms and the degree of impairment in daily functioning
• Whether psychiatric symptoms such as depression, anxiety, or sleep problems are present

Examples of questions to submit to or prepare for the doctor

• What restrictions (such as food or medication discontinuation) must I follow for the scheduled tests (late-night salivary cortisol, 24-hour urine test, dexamethasone suppression test, etc.)?
• Do my current medications (especially antifungal drugs, antidepressants, etc.) affect the test results?

General Example Questions to Ask the Doctor

• What do you think is causing my condition? (Exogenous, pituitary, adrenal, or ectopic)
• What additional tests do I need, and what is the purpose of each?
• What do you recommend as the first-line treatment in my case (surgery or medication)? What are the expected benefits and risks?
• What are the major adverse effects of the available medications?
• What will the plan be for steroid replacement therapy after surgery or for long-term follow-up?
• What lifestyle changes do you recommend to reduce complications (cardiovascular disease, osteoporosis, diabetes, etc.)?

Examples of Disease-Specific Questions

• My ACTH test result is low (or high). In this case, is testing of the adrenal glands (or the pituitary gland/ectopic source) essential? What imaging should be recommended as the next step?
• I am scheduled for surgery. What is the plan for steroid administration (replacement therapy) before and after surgery, and what symptoms should I watch for during hospitalization?

(Medical decisions should be made based on examination by the treating specialist and test results, and the indicated tests may be selected or changed depending on the individual’s condition.)