Autoimmune polyendocrine syndrome medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

OR

The majority of cases of [disease name] are self-limited and require only supportive care.

OR

[Disease name] is a medical emergency and requires prompt treatment.

OR

The mainstay of treatment for [disease name] is [therapy].

OR   The optimal therapy for [malignancy name] depends on the stage at diagnosis.

OR

[Therapy] is recommended among all patients who develop [disease name].

OR

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

OR

Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

OR

Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

OR

Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Medical Therapy

Medical therapy for autoimmune polyendocrine syndrome (APS) depends upon the subtype and the organ system involved.

• In APS the focus is to treat the presenting disorder which can either be mucocutaneous candidiasis, hypoparathyroidism, adrenal insufficiency or autoimmune thyroiditis.
• Patient education is an important aspect of treatment in APS. Patient with APS who present with mucocutaneous candidiasis may take upto 5-10 years to develop hypoparathyroidism and another 10 years to show manifestations of adrenal insufficiency. Therefore, patients with family history of APS or an early age onset of APS should be advised to undergo regular surveillance.
• Treatment of APS 1 includes:
  • Mucocutaneous candidiasis:
    • Preferred regimen(1): Fluconazole 800 mg (12 mg/kg) on day 1, then 400 mg daily (6 mg/kg/day) for 14 days after first negative blood culture and resolution of signs/symptoms
    • Alternative regimen (1): Ketoconazole 200-400 mg/day PO may increase to 400 mg once daily if response is insufficient. Continue until active fungal infection is resolved; some infections may require a treatment duration of up to 6 months.
  • Hypoparathyroidism: Conventional therapy for hypoparathyroidism:
    • Oral calcium: Preferred regimen (1): Calcium carbonate (40% elemental calcium) (better absorption with meals)

      • Alternative regimen (1): Calcium citrate (21% elemental calcium) (more effective in patients with achlorhydria and proton pump inhibitors' use, worsening constipation)

    • Vitamin D supplementation

      • Preferred regimen (1): Calcitriol 0.25 to 2 μg q24h (>.75 μg administered in divided doses)
      • Preferred regimen (2): Cholecalciferol (parent vitamin D3)
      • Preferred regimen (3): Ergocalciferol (parent vitamin D2)
      • Alternative regimen (1): 1α-Hydroxyvitamin D (alfacalcidol) (used outside the United States)
      • Alternative regimen (2): Dihydrotachysterol (used outside the United States)

      Note(1): Serum calcium (corrected for albumin), phosphorus, and creatinine concentrations should be measured weekly to monthly during dose adjustments, and twice annually once a stable regimen has been reached.

      Note(2): 24 Hour urinary calcium and creatinine should be considered during dose adjustments and should be measured twice annually on a stable regimen to evaluate for renal toxicity.

  • Adrenal insufficiency (Addison's disease):
    • Glucocorticosteroid: Preferred regimen (1): Cortisone 10 to 37.5 mg q12h orally given in 2 divided doses with two-thirds of the total dose given in the morning and one third in the afternoon

      • Preferred regimen (2): Hydrocortisone : 15-30 mg/day orally given in 2 divided doses with two-thirds of the total dose given in the morning and one third in the afternoon
      • Preferred regimen (3): Dexamethasone : 0.25 to 0.75 mg orally once daily
      • Preferred regimen (4): Prednisone : 2.5 to 5 mg orally once daily

    • Mineralocorticosteroid: Preferred regimen (1): Fludrocortisone : 0.1 to 0.2 mg orally once daily
    • Patients of Addison's disease with mild-to-moderate stress:

      • Alternative regimen (1): Cortisone 50-100 mg/day orally given in 2 divided doses with two-thirds of the total dose given in the morning (around 8 a.m.) and one third in the afternoon (noon to 4 p.m.) for 3 days
      • Alternative regimen (2): Hydrocortisone 30-90 mg/day orally given in 2 divided doses with two-thirds of the total dose given in the morning (around 8 a.m.) and one third in the afternoon (noon to 4 p.m.) for 3 days
      • Alternative regimen (3): Dexamethasone 0.50 to 2.25 mg orally once daily for 3 days
      • Alternative regimen (4): Prednisone 5-15 mg orally once daily for 3 days

    • Patients of Addison's disease with severe stress:

      • Alternative regimen (5): Hydrocortisone sodium succinate 100 mg intravenously every 6-8 hours

  • Autoimmune thyroiditis:

    • • Preferred regimen (1) Synthetic levothyroxine (L-T4) 1.6–1.8 μg/kg of body weight per day orally.

• APS type 1: Chronic mucocutaneous candidiasis is commonly present in patients with APS type 1 and should be aggressively treated to prevent developmeent of epithelial carcinoma.

In addition the medical therapy should be aimed at the autoimmune disorder present.

Type I: Treatments focus on the three major clinical features, including fluconazole and ketoconazole, calcium and vitamin D, and steroid. The prognosis of autoimmune polyendocrine syndrome type I is variable, depending on how organs are affected and the severity of the disease.

Type II: Treatment involves drug therapy. Drugs for such signs mentioned above are the main treatment opinion, including replacement therapy with hydrocortisone and fludrocortisone, antithyroid medications, insulin, and medicines on other manifestations. Prognosis of autoimmune polyendocrine syndrome type II depends on whether endocrine end-organ failures occur or not.

Type III: Compared with type I and II, autoimmune polyendocrine syndrome type III does not involve the adrenal cortex. Studies demonstrate that autoimmunity, environmental factors and genetic factors are involved in the cause of autoimmune polyendocrine syndrome type III. The goals of treatment of autoimmune polyendocrine syndrome type III are to correct hormone deficiencies, prevent complications, and reduce morbidity. Treatments include monitoring of glandular functions for early detection of glandular failure, lifelong hormone replacement therapy for established glandular failure, and familial screening.