Type I Polyglandular Autoimmune Syndrome Treatment & Management

Updated: Nov 11, 2021
  • Author: Saleh A Aldasouqi, MD, FACE, ECNU; Chief Editor: Romesh Khardori, MD, PhD, FACP  more...
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Treatment

Medical Care

The treatment for polyglandular autoimmune (PGA) syndrome, type I, is targeted at 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

  • This condition is treated with oral fluconazole and ketoconazole.

  • Absorption of ketoconazole may be compromised if coexistent atrophic gastritis exists. Ketoconazole may also inhibit adrenal and gonadal synthesis, which could worsen the coexistent Addison disease and cause hepatitis.

  • Fluconazole is preferred, because it does not inhibit steroidogenesis and is less frequently associated with the development of hepatitis. It is, however, an expensive medication.

  • Treatment of oral candidiasis is indicated in order to prevent the late complication of epithelial carcinoma.

Hypoparathyroidism

  • This disorder 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/day. Calcitriol (1,25-dihydroxy D) is a better choice physiologically, but it is more expensive. Other vitamin D synthetic analogues also are suitable for replacement, but cost again must be considered.

  • In cases in which there is coexisting malabsorption, tetany may occur and IV calcium gluconate and magnesium may be necessary.

  • The hypocalcemia seen in PGA-I also has been reported to result from pancreatic insufficiency, giardiasis (which occurs with increased frequency in PGA-I), and lymphangiectasia. Each of these requires specific therapy.

Adrenal insufficiency (Addison disease)

  • The treatment of adrenal failure depends mainly on 2 factors.

    • Treatment is influenced by the question of whether or not the patient is in crisis with hypotension and consequently requires IV fluids and IV steroids. Otherwise, treatment is influenced by the question of whether or not chronic and otherwise stable oral steroids, eg, prednisone, can be used with or without fludrocortisone.

    • Another factor influencing treatment is whether or not a confident diagnosis of adrenal failure can be made based on the information at hand when the patient is seen. 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.

  • 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.

  • In cases of intercurrent illness, increase the doses of hydrocortisone.

  • In the presence of coexisting diabetes, which is 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.

  • Other deficiencies seen in association with diabetes and pernicious anemia, eg, hypothyroidism, can be corrected by replacement therapy.

  • Adrenal gland transplants have been successful in experimental rodents and in humans.

  • Vitamin and mineral replacement occasionally is needed to complement hormonal replacement.

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Surgical Care

No specific surgical interventions exist that are unique to the management of polyglandular autoimmune (PGA) syndrome, type I. However, complications from a component of the syndrome may require therapeutic procedures or surgical interventions, as for example, in the case of a patient requiring intubation and other critical care therapeutic interventions after going into adrenal crisis culminating in septic/hypovolemic shock.

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Consultations

See the list below:

  • Endocrinology - Complex interactions exist that may affect the replacement of adrenal, thyroid, and parathyroid hormones; these are best handled by an endocrinologist.

  • Infectious diseases - To help with recurrent candidiasis

  • Gastroenterology - If bowel or hepatic involvement is noted

  • Rheumatology - If necessary because of the autoimmune nature of the disease, especially when considering immunosuppressive therapy

  • Other consultations may be needed according to the clinical situation.

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Diet

A high-salt diet is beneficial to patients with adrenal insufficiency.

If coexisting diabetes is present, institute a diabetic diet.

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Activity

As tolerated.

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