Autoimmune polyendocrine syndrome medical therapy

Autoimmune polyendocrine syndrome Microchapters
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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

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.

Medical Therapy

• Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
• Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
• Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
• Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Disease Name

• 1 Stage 1 - Name of stage
  • 1.1 Specific Organ system involved 1
    • 1.1.1 Adult
      • Preferred regimen (1): drug name 100 mg PO q12h for 10-21 days (Contraindications/specific instructions)
      • Preferred regimen (2): drug name 500 mg PO q8h for 14-21 days
      • Preferred regimen (3): drug name 500 mg q12h for 14-21 days
      • Alternative regimen (1): drug name 500 mg PO q6h for 7–10 days
      • Alternative regimen (2): drug name 500 mg PO q12h for 14–21 days
      • Alternative regimen (3): drug name 500 mg PO q6h for 14–21 days
    • 1.1.2 Pediatric
      • 1.1.2.1 (Specific population e.g. children < 8 years of age)
        • Preferred regimen (1): drug name 50 mg/kg PO per day q8h (maximum, 500 mg per dose)
        • Preferred regimen (2): drug name 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
        • Alternative regimen (1): drug name10 mg/kg PO q6h (maximum, 500 mg per day)
        • Alternative regimen (2): drug name 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
        • Alternative regimen (3): drug name 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
      • 1.1.2.2 (Specific population e.g. 'children < 8 years of age')
        • Preferred regimen (1): drug name 4 mg/kg/day PO q12h（maximum, 100 mg per dose）
        • Alternative regimen (1): drug name 10 mg/kg PO q6h (maximum, 500 mg per day)
        • Alternative regimen (2): drug name 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
        • Alternative regimen (3): drug name 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
  • 2.1 Specific Organ system involved 2
    • 2.1.1 Adult
      • Preferred regimen (1): drug name 500 mg PO q8h
    • 2.1.2 Pediatric
      • Preferred regimen (1): drug name 50 mg/kg/day PO q8h (maximum, 500 mg per dose)

• 2 Stage 2 - Name of stage
  • 2.1 Specific Organ system involved 1

    Note (1):

    Note (2):

    Note (3):

    • 2.1.1 Adult
      • Parenteral regimen
        • Preferred regimen (1): drug name 2 g IV q24h for 14 (14–21) days
        • Alternative regimen (1): drug name 2 g IV q8h for 14 (14–21) days
        • Alternative regimen (2): drug name 18–24 MU/day IV q4h for 14 (14–21) days
      • Oral regimen
        • Preferred regimen (1): drug name 500 mg PO q8h for 14 (14–21) days
        • Preferred regimen (2): drug name 100 mg PO q12h for 14 (14–21) days
        • Preferred regimen (3): drug name 500 mg PO q12h for 14 (14–21) days
        • Alternative regimen (1): drug name 500 mg PO q6h for 7–10 days
        • Alternative regimen (2): drug name 500 mg PO q12h for 14–21 days
        • Alternative regimen (3):drug name 500 mg PO q6h for 14–21 days
    • 2.1.2 Pediatric
      • Parenteral regimen
        • Preferred regimen (1): drug name 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
        • Alternative regimen (1): drug name 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
        • Alternative regimen (2):  drug name 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day) '(Contraindications/specific instructions)'
      • Oral regimen
        • Preferred regimen (1): drug name 50 mg/kg/day PO q8h for 14 (14–21) days (maximum, 500 mg per dose)
        • Preferred regimen (2): drug name (for children aged ≥ 8 years) 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
        • Preferred regimen (3): drug name 30 mg/kg/day PO q12h for 14 (14–21) days (maximum, 500 mg per dose)
        • Alternative regimen (1): drug name 10 mg/kg PO q6h 7–10 days (maximum, 500 mg per day)
        • Alternative regimen (2): drug name 7.5 mg/kg PO q12h for 14–21 days (maximum, 500 mg per dose)
        • Alternative regimen (3): drug name 12.5 mg/kg PO q6h for 14–21 days (maximum,500 mg per dose)
  • 2.2 'Other Organ system involved 2'

    Note (1):

    Note (2):

    Note (3):

    • 2.2.1 Adult
      • Parenteral regimen
        • Preferred regimen (1): drug name 2 g IV q24h for 14 (14–21) days
        • Alternative regimen (1): drug name 2 g IV q8h for 14 (14–21) days
        • Alternative regimen (2): drug name 18–24 MU/day IV q4h for 14 (14–21) days
      • Oral regimen
        • Preferred regimen (1): drug name 500 mg PO q8h for 14 (14–21) days
        • Preferred regimen (2): drug name 100 mg PO q12h for 14 (14–21) days
        • Preferred regimen (3): drug name 500 mg PO q12h for 14 (14–21) days
        • Alternative regimen (1): drug name 500 mg PO q6h for 7–10 days
        • Alternative regimen (2): drug name 500 mg PO q12h for 14–21 days
        • Alternative regimen (3):drug name 500 mg PO q6h for 14–21 days
    • 2.2.2 Pediatric
      • Parenteral regimen
        • Preferred regimen (1): drug name 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
        • Alternative regimen (1): drug name 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
        • Alternative regimen (2):  drug name 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day)
      • Oral regimen
        • Preferred regimen (1): drug name 50 mg/kg/day PO q8h for 14 (14–21) days (maximum, 500 mg per dose)
        • Preferred regimen (2): drug name 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
        • Preferred regimen (3): drug name 30 mg/kg/day PO q12h for 14 (14–21) days (maximum, 500 mg per dose)
        • Alternative regimen (1): drug name 10 mg/kg PO q6h 7–10 days (maximum, 500 mg per day)
        • Alternative regimen (2): drug name 7.5 mg/kg PO q12h for 14–21 days (maximum, 500 mg per dose)
        • Alternative regimen (3): drug name 12.5 mg/kg PO q6h for 14–21 days (maximum,500 mg per dose)