Adrenal insufficiency

Disease Description (Causes and Pathophysiology, Main Symptoms)

• Summary of definition: A condition in which secretion of cortisol (glucocorticoid) and aldosterone (mineralocorticoid) from the adrenal cortex is insufficient. Cortisol deficiency causes problems with energy metabolism, the stress response, and blood pressure regulation, while aldosterone deficiency causes problems with sodium, potassium, and fluid balance.
• Main causes
  • Primary (abnormality of the adrenal glands themselves): autoimmune adrenalitis (most common in developed countries), tuberculosis (important in areas where it occurs), metastatic cancer, vascular injury (hemorrhage), fungal infection, hereditary causes (congenital adrenal hypoplasia, adrenoleukodystrophy), etc.
  • Secondary (abnormalities of the hypothalamus or pituitary gland, or after long-term administration of exogenous steroids): pituitary insufficiency, suppression of the HPA axis caused by long-term glucocorticoid use, etc.
• Pathophysiological features
  • Primary: cortisol↓, aldosterone↓ → ACTH↑ (possible skin hyperpigmentation), hyponatremia, hyperkalemia, hypotension, etc.
  • Secondary: ACTH↓ → cortisol↓, but aldosterone is regulated mainly by the renin–angiotensin system and is therefore often preserved, so hyperkalemia is uncommon; no skin hyperpigmentation.
• Main symptoms
  • Chronic: fatigue and lethargy, loss of appetite and weight loss, nausea, vomiting, and abdominal pain, hypotension (orthostatic hypotension), hypoglycemia (especially in children), skin hyperpigmentation (primary), salt craving, decreased body hair, etc.
  • Acute (adrenal crisis): severe vomiting and abdominal pain, high fever, hypotension and shock, decreased consciousness, severe electrolyte abnormalities (low sodium and high potassium), hypoglycemia — emergency treatment is required.

Treatment Methods (Standard Treatment, Medications, Procedures/Surgery, Lifestyle Management and Precautions)

• Chronic (basic principles)
  • Glucocorticoid replacement: oral hydrocortisone 15–25 mg/day, usually divided between immediately after waking and lunchtime (e.g., 10 mg in the morning, 5 mg at noon, and 5 mg in the afternoon), or replacement with prednisone/prednisolone.
  • For aldosterone deficiency (in primary cases), mineralocorticoid replacement: fludrocortisone 0.05–0.2 mg/day (with individualized dose adjustment).
  • Salt intake is recommended (especially in primary cases), along with monitoring of blood pressure and electrolytes.
• Adrenal crisis (emergency)
  • Immediately administer intravenous hydrocortisone 100 mg as a bolus, followed by 200 mg/24 hours (intermittent injections or continuous infusion), or 50–100 mg IV q6–8h.
  • Replenish blood pressure and body fluids with large amounts of isotonic fluid (normal saline); administer glucose in cases of hypoglycemia.
  • Correct electrolyte abnormalities and treat the cause (such as infection) at the same time.
• Additional treatment by cause
  • When there is an underlying infection such as tuberculosis, administer the relevant antituberculosis or antimicrobial drugs concurrently (steroid dose adjustment is necessary).
  • If the cause is a tumor, hemorrhage, or similar condition, consider surgical, radiation, or anticancer treatment.
  • In secondary cases, perform brain imaging, provide hormone replacement, and treat the underlying pituitary disease.
• Lifestyle management and education
  • “Sick day rules”: Temporarily increase the glucocorticoid dose during stress such as febrile illness, surgery, trauma, diarrhea, or vomiting (as directed by a doctor).
  • Keeping an emergency hydrocortisone self-injection kit is recommended, and instruction on its use should be provided.
  • Wearing medical identification (an emergency card or bracelet) is recommended.
  • Regular testing of blood pressure, body weight, and electrolytes (sodium and potassium).
• Complications and precautions
  • Inadequate treatment increases the risk of recurrent adrenal crises, chronic weakness, hypoglycemia, and electrolyte abnormalities.
  • Excessive dosing can cause steroid-related adverse effects (weight gain, osteoporosis, increased susceptibility to infection, hyperglycemia, etc.).
  • Dose adjustment is necessary during pregnancy, surgery, or severe infection — make a plan in advance with medical staff.

Outpatient Visit Checkpoints (Items to Prepare and Example Questions)

• Items to prepare (recommended to bring)
  • A list of medications and supplements currently being taken (including doses and administration times).
  • Recent blood test results (electrolytes, fasting blood glucose, serum cortisol, ACTH, etc.) or test records.
  • Recent hospital records (history of hospitalization, surgery, infection, or tuberculosis).
  • Notes on changes in symptoms (when they began and in what situations they worsen), and records of weight and blood pressure measurements.
  • An emergency hydrocortisone prescription (if available) and information on whether an emergency injection kit is in your possession.
• Prepare in advance the items that a doctor is likely to ask about during an outpatient visit
  • Recent fever, diarrhea, vomiting, or infection; whether surgery is planned; possibility of pregnancy or whether you are pregnant; history of long-term steroid use.
• Example questions (sentences the patient can prepare in advance)
  • “What were my cortisol/ACTH levels in the recent tests?”
  • “Are there any medications I am currently taking that need to be adjusted?”

General Example Questions to Ask the Doctor

• What are the expected adverse effects of this replacement therapy, and how will they be monitored?
• Specifically, how should I increase my medication dose during stressful situations such as fever, surgery, or a traffic accident?
• Do I need aldosterone (fludrocortisone) replacement?
• How should my family respond if an adrenal crisis (emergency condition) occurs?
• What regular follow-up tests are needed (which tests and how often)?

Examples of Disease-Specific Questions

• (If primary disease is suspected or diagnosed) What additional tests should be performed to determine whether the cause of my disease is autoimmune, tuberculosis or another infection, or cancer? (Examples: 21-hydroxylase antibodies, adrenal CT)
• (If there is a history of long-term steroid use) Suppression of the HPA axis due to previous steroid use is suspected. When is the appropriate time to perform tests to determine whether recovery has occurred (for example, an ACTH stimulation test)?

(If necessary, presenting test result sheets, medication records, and a photo of the emergency kit together during a consultation with the doctor can help establish a diagnosis and treatment plan.)