Autoimmune Polyglandular Syndrome, Type II

Aka: Autoimmune Polyglandular Syndrome, Type II, Autoimmune Polyglandular Syndrome, APS II

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II. Epidemiology

  1. Prevalence: 2 per 100,000
  2. Age of Onset: 30-40 years
  3. Gender: Women affected 3 times more than men

III. Risk Factors

  1. HLA haplotype DR3 (DQB*0201)
  2. HLA haplotype DR4 (DQB1*0302)

IV. Diagnosis

  1. Required Conditions for diagnosis
    1. Autoimmune Adrenal Insufficiency
      1. Presenting diagnosis in 50% of cases
      2. Associated with second Autoimmune Condition in 50%
    2. Autoimmune Thyroid disease
    3. Type I Diabetes Mellitus
  2. Commonly Associated Conditions (4-10%)
    1. Vitiligo
    2. Chronic atrophic Gastritis (and Pernicious Anemia)
    3. Hypergonadotropic Hypogonadism
    4. Chronic Autoimmune Hepatitis
    5. Alopecia
  3. Other Associated Conditions (<1%)
    1. Myasthenia Gravis
    2. Rheumatoid Arthritis
    3. Sjogren's Syndrome

V. Presentation

  1. See Adrenal Insufficiency
  2. See other associated conditions listed above

VI. Diagnosis

  1. See Adrenal Insufficiency
  2. See other associated conditions listed above

VII. Management

  1. See Adrenal Insufficiency
  2. See other associated conditions listed above

VIII. References

  1. Betterie (2002) Endocr Rev 23:327-64 [PubMed]
  2. Majeroni (2007) Am Fam Physician 75(5):667-70 [PubMed]

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