IT IS USEFUL FOR SCREENING FAMILY MEMBERS WHO MAY DEVELOP AUTOIMMUNE ENDOCRINE DISEASE IN FUTURE.
Medical Care
Treatment is targeted to whatever organ is affected. It is always best to identify and treat the respective autoimmunity before any significant morbidity can develop.
For the most part, replacement therapy and patient education about the chronic diseases are integral to treatment success. The educational aspect is extremely important because it helps the patient with the early detection of any new autoimmune states and aids in the adequate treatment of this chronic syndrome.
" Mucocutaneous candidiasis
o It is treated with oral fluconazole and ketoconazole.
o Absorption of ketoconazole may be compromised if coexistent atrophic gastritis exists. Ketoconazole also may inhibit adrenal and gonadal synthesis, which could worsen the coexistent Addison disease and cause hepatitis.
o Fluconazole is preferred because it does not inhibit steroidogenesis and has a less frequent occurrence of hepatitis. However, it is expensive.
" Hypoparathyroidism
o It usually is gradual and permanent, and oral calcium and vitamin D usually are adequate therapy. Doses of vitamin D range from 50,000-100,000 U/d. Calcitriol (1,25-dihydroxy D) is a better choice physiologically, but it is more expensive. Other newer vitamin D synthetic analogues are also suitable for replacement, but cost is to be again considered.
o In cases with coexisting malabsorption, tetany may occur and IV calcium gluconate and magnesium may be necessary.
o The hypocalcemia seen in PGA-I also has been reported to be from pancreatic insufficiency, giardiasis (which occurs with increased frequency in PGA-I), and lymphangiectasia. Each of these requires specific therapy.
" Adrenal insufficiency (Addison disease)
o The treatment of adrenal failure mainly depends on 2 things.
" Whether the patient is in crises with hypotension and, as such, requires IV fluids and IV steroids influences treatment. Otherwise, whether chronic and otherwise stable oral steroids, eg, prednisone, can be used with or without fludrocortisone influences treatment.
" Whether a confident diagnosis of adrenal failure can be made based on the information at hand when the patient is seen is another factor influencing treatment. This may determine what kind of IV steroid is used. If the diagnosis is not clear, then the physician may opt to use dexamethasone IV because it does not interfere with subsequent cortisol measurements required for the diagnosis of Addison disease. However, if sufficient clinical evidence exists in favor of Addison disease, then using hydrocortisone is better because of its additional mineralocorticoid benefit, as an aldosterone defect also is seen. Most of the time, a mineralocorticoid (eg, fludrocortisone) also is added to the regimen.
o The glucocorticoid dose is changed according to the patient's symptoms. Monitor electrolytes and the activity levels of plasma renin to assess the efficacy of treatment with fludrocortisone.
o In cases of intercurrent illness, increase the doses of hydrocortisone.
o In the presence of coexisting diabetes occasionally seen with PGA-I, the daily dose usually should not exceed 30 mg/d, unless the need for a larger dosage is confirmed. This necessitates higher doses of insulin; on many occasions, this results in difficulty controlling glucose levels.
o Other deficiencies seen in association with diabetes and pernicious anemia, eg, hypothyroidism, can be corrected by replacement therapy.
o Adrenal gland transplants have been successful in experimental rodents and humans.
o Vitamin and mineral replacement occasionally is needed to compliment hormonal replacement.
Surgical Care
No specific surgical interventions exist that are uniquely needed in the management of the PGA-I syndrome. However, complications from a component of the syndrome may require therapeutic procedures or surgical interventions, as appropriate. For example, a patient going into adrenal crisis culminating in septic/hypovolemic shock and requiring intubation and other critical care therapeutic interventions.
Diet
" A high-salt diet is beneficial to patients with adrenal insufficiency.
" If coexisting diabetes is present, institute a diabetic diet.
Activity
As tolerated
DRUG TREATMENT :
1. Corticosteroids : Used for adrenocortical insufficiency replacement. These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.
- HYDROCORTISONE
- FLUDROCORTISONE
2. ANTIFUNGALS :
- KETOCONAZOLE
- FLUCONAZOLE
3. VITAMINS & MINERALS
- CALCITRIOL
- ERGOCALCIFEROL
- CALCIUM CARBONATE