UpToDate 2017 Type I AIPGS: adrenal insufficiency, hypoparathyroidism, mucocutaneous candidiasis. Type II AIPGS: adrenal insufficiency, autoimmune thyroid disease, and DM1.
Diabetes insipidus is often
present. CMDT 2017 UpToDate 2017 Symptom Frequency (%) Weakness, tiredness, fatigue 100 Anorexia 100 Gastrointestinal symptoms 92 - Nausea 86 - Vomiting 75 - Constipation 33 - Abdominal pain 31 - Diarrhea 16 Salt craving 16 UpToDate Postural dizziness 12 2017 Muscle or joint pains 6-13 Sign Frequency (%) Weight loss 100 Hyperpigmentation 94 Hypotension (<110 mm Hg systolic) 88-94 Vitiligo 10-20 Auricular calcification 5 UpToDate 2017 Laboratory Finding Frequency (%) Electrolyte disturbances 92 - Hyponatremia 88 - Hyperkalemia 64 - Hypercalcemia 6 Azotemia 55 UpToDate Anemia 40 2017 Eosinophilia 17 IAP Vs. IAST IAST: no hiperpigmentacin, no deshidratacin, no hiperkalemia, hipotensin menos prominente, sntomas GI menos comunes. La hipoglucemia es ms comn en la IAST. UpToDate 2017 EMERGENCY MEASURES Draw blood for serum electrolytes, glucose, and plasma cortisol and ACTH. Infuse 2-3 L of NS or 5% Dw. Inject IV hydrocortisone (100 mg immediately and every 6-8 hr) Use supportive measures as needed. SUBACUTE MEASURES AFTER STABILIZATION OF THE PATIENT Continue intravenous NS at a slower rate for next 24-48h. Search for and treat possible infectious precipitating causes of the adrenal crisis. Perform a short ACTH stimulation test to confirm the diagnosis of adrenal insufficiency, if patient does not have known adrenal insufficiency. Determine the type of adrenal insufficiency and its cause if not already known. Taper glucocorticoids to maintenance dosage over 1-3 days, if precipitating or complicating illness permits. Begin mineralocorticoid replacement with fludrocortisone (0.1 mg by mouth daily) when saline infusion is stopped. MAINTENANCE THERAPY Glucocorticoid Replacement Hydrocortisone 15-20 mg on awakening and 5-10 mg in early afternoon. Monitor clinical symptoms and morning plasma ACTH. Mineralocorticoid Replacement Fludrocortisone 0.1 (0.05-0.2) mg orally. Liberal salt intake. Monitor lying and standing blood pressure and pulse, edema, serum potassium, and plasma renin activity. Educate patient about the disease, how to manage minor illnesses and major stresses, and how to inject steroid intramuscularly. TREATMENT OF MINOR FEBRILE ILLNESS OR STRESS Increase glucocorticoid dose 2-fold to 3-fold for the few days of illness; do not change mineralocorticoid dose. No extra supplementation is needed for most uncomplicated, outpatient dental procedures under local anesthesia. General anesthesia or intravenous sedation should not be used in the office.