Adrenal

Autoimmune Polyglandular Syndrome, Type II

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Autoimmune Polyglandular Syndrome, Type II, Autoimmune Polyglandular Syndrome, APS II

  • See Also
  1. Autoimmune Polyglandular Syndrome, Type I
  • Epidemiology
  1. Prevalence: 2 per 100,000
  2. Age of Onset: 30-40 years
  3. Gender: Women affected 3 times more than men
  • Risk Factors
  1. HLA haplotype DR3 (DQB*0201)
  2. HLA haplotype DR4 (DQB1*0302)
  • 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
  • Presentation
  1. See Adrenal Insufficiency
  2. See other associated conditions listed above
  • Diagnosis
  1. See Adrenal Insufficiency
  2. See other associated conditions listed above
  • Management
  1. See Adrenal Insufficiency
  2. See other associated conditions listed above
  • References
  1. Betterie (2002) Endocr Rev 23:327-64 [PubMed]
  2. Majeroni (2007) Am Fam Physician 75(5):667-70 [PubMed]