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

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

Polyendocrinopathies, Autoimmune (C0085409)

Definition (MSH)Autoimmune diseases affecting multiple endocrine organs. Type I is characterized by childhood onset and chronic mucocutaneous candidiasis (CANDIDIASIS, CHRONIC MUCOCUTANEOUS), while type II exhibits any combination of adrenal insufficiency (ADDISON'S DISEASE), lymphocytic thyroiditis (THYROIDITIS, AUTOIMMUNE;), HYPOPARATHYROIDISM; and gonadal failure. In both types organ-specific ANTIBODIES against a variety of ENDOCRINE GLANDS have been detected. The type II syndrome differs from type I in that it is associated with HLA-A1 and B8 haplotypes, onset is usually in adulthood, and candidiasis is not present.
ConceptsDisease or Syndrome (T047)
ICD9258.1
MSHD016884
EnglishAPS, Autoimmune Polyendocrinopathies, Autoimmune polyendocrinopathy, Autoimmune polyglandular failure, Autoimmune polyglandular syndrome, Lloyd's syndrome, PGA, Polyendocrine autoimmunity syndrome, Polyglandular autoimmune syndrome
Spanishpoliendocrinopatia autoinmune, sindrome APS, sindrome autoinmune poliglandular, sindrome de Lloyd, sindrome PGA, sindrome poliglandular autoinmune
Parent ConceptsAutoimmune Diseases (C0004364), Endocrine System Diseases (C0014130), Polyglandular dysfunction NOS (C0154222), Autoimmune endocrine disease (C0342552), Reason not stated concept (C1276325), Duplicate concept (C1274013)
SourcesMSH, MTH, MTHICD9, NDFRT, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)


Autoimmune Syndrome Type II, Polyglandular (C0085860)

ConceptsDisease or Syndrome (T047)
ICD9258.1
MSHD016884
EnglishAddison's disease with struma lymphomatosa, APS II, APS type 2, APS2, Autoimmune Polyglandular Syndrome Type II, PGA - Polyglandular autoimmune syndrome - type II, PGA II, Polyglandular autoimmune syndrome - type II, Polyglandular Type II Autoimmune Syndrome, Primary hypothyroidism AND adrenocortical insufficiency, Schmidt Syndrome, Schmidt's syndrome, Schmidts Syndrome, Type 2 polyendocrine autoimmunity syndrome
SpanishAPS tipo 2, enfermedad de Addison con estruma linfomatoso, hipotiroidismo primario e insuficiencia adrenocortical, hipotiroidismo primario Y enfermedad de Addison, sindrome de Schmidt
Parent ConceptsDiabetes Mellitus, Insulin-Dependent (C0011854), Graves' Disease (C0018213), Polyendocrinopathies, Autoimmune (C0085409), Diabetes mellitus associated with genetic syndrome (C0342274), Reason not stated concept (C1276325)
SourcesCSP, MSH, MTH, MTHICD9, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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