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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Related Studies

Ontology: Polyendocrinopathies, Autoimmune (C0085409)

Definition (CHV) an autoimmune diseases affecting multiple endocrine organs
Definition (CHV) an autoimmune diseases affecting multiple endocrine organs
Definition (CHV) an autoimmune diseases affecting multiple endocrine organs
Definition (CHV) an autoimmune diseases affecting multiple endocrine organs
Definition (NCI) A rare genetic syndrome characterized by autoantibodies production against more than one endocrine organ. The range of endocrinopathies includes hypoparathyroidism, adrenal insufficiency, hypogonadism, type 1 diabetes mellitus and hypothyroidism.
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.
Concepts Disease or Syndrome (T047)
MSH D016884
ICD10 E31.0
SnomedCT 18947001, 190567009, 190568004, 41864002
English Polyendocrinopathy, Autoimmune, Autoimm polyglandular failure, Polyendocrine autoimmunity syn, Lloyd's syndrome (diagnosis), polyendocrine failure syndrome (diagnosis), endocrine Lloyd's syndrome, polyendocrine failure syndrome, Autoimmune Polyendocrinopathy, Autoimmune Polyendocrinopathy Syndrome, Polyendocrinopathies, Autoimmune [Disease/Finding], lloyd's syndrome, autoimmune polyendocrinopathy, pga, aps, autoimmune polyendocrine syndrome (diagnosis), autoimmune polyendocrine syndrome, Lloyd's syndrome (disorder), Autoimmune polyglandular failure (disorder), PGA, APS, Lloyd's syndrome, Autoimmune polyglandular failure, Polyendocrine autoimmunity syndrome, Polyglandular autoimmune syndrome, Autoimmune polyendocrinopathy (disorder), Autoimmune polyendocrinopathy, Autoimmune polyglandular syndrome, autoimmune; polyglandular syndrome, deficiency; polyglandular, autoimmune, polyglandular; deficiency, autoimmune, polyglandular; syndrome, autoimmune, syndrome; autoimmune polyglandular, syndrome; polyglandular, autoimmune, Autoimmune polyendocrinopathy, NOS, Autoimmune polyglandular syndrome, NOS, Polyglandular autoimmune syndrome, NOS, Polyendocrinopathies, Autoimmune
Swedish Polyendokrinopatier, autoimmuna
Czech polyendokrinopatie autoimunitní
Finnish Autoimmuunipolyendokrinopatiat
Russian SHMIDTA SINDROM, POLIENDOKRINOPATII AUTOIMMUNNYE, AUTOIMMUNNYI SINDROM I TIPA POLIGLANDULIARNYI, AUTOIMMUNNYI SINDROM II TIPA POLIGLANDULIARNYI, POLIGLANDULIARNYI I TIPA AUTOIMMUNNYI SINDROM, AUTOIMMUNNAIA POLIENDOKRINOPATIIA-KANDIDOZ-EKTODERMAL'NAIA DISTROFIIA, POLIGLANDULIARNYI II TIPA AUTOIMMUNNYI SINDROM, ENDOKRINNYE BOLEZNI MNOZHESTVENNYE AUTOIMMUNNYE, АУТОИММУННАЯ ПОЛИЭНДОКРИНОПАТИЯ-КАНДИДОЗ-ЭКТОДЕРМАЛЬНАЯ ДИСТРОФИЯ, АУТОИММУННЫЙ СИНДРОМ II ТИПА ПОЛИГЛАНДУЛЯРНЫЙ, АУТОИММУННЫЙ СИНДРОМ I ТИПА ПОЛИГЛАНДУЛЯРНЫЙ, ПОЛИГЛАНДУЛЯРНЫЙ II ТИПА АУТОИММУННЫЙ СИНДРОМ, ПОЛИГЛАНДУЛЯРНЫЙ I ТИПА АУТОИММУННЫЙ СИНДРОМ, ПОЛИЭНДОКРИНОПАТИИ АУТОИММУННЫЕ, ШМИДТА СИНДРОМ, ЭНДОКРИННЫЕ БОЛЕЗНИ МНОЖЕСТВЕННЫЕ АУТОИММУННЫЕ
German Autoimmune polyglandulaere Insuffizienz, Polyendokrinopathien, Autoimmun-
Japanese シュミット症候群, 自己免疫性多発性内分泌症, 多腺性自己免疫症候群, 自己免疫性多発性内分泌症-カンジダ症-外胚葉ジストロフィー, 多腺性自己免疫症候群1型, 多腺性2型自己免疫症候群, 自己免疫症候群2型-多腺性, 自己免疫症候群1型-多腺性, 多腺性自己免疫症候群2型, 多腺性1型自己免疫症候群, Schmidt症候群, 多発性内分泌症-自己免疫性
Korean 자가면역성 뭇샘성 기능상실
Spanish poliendocrinopatía autoinmunitaria (trastorno), poliendocrinopatía autoinmune (trastorno), poliendocrinopatía autoinmune, poliendocrinopatía autoinmunitaria, síndrome autoinmune poliglandular, síndrome poliglandular autoinmune, síndrome de Lloyd (concepto no activo), síndrome de Lloyd, síndrome APS, síndrome PGA, Poliendocrinopatías Autoinmunes
Polish Choroby gruczołów dokrewnych autoimmunologiczne, Zespół Schmidta, Zespół Carpentera, Autoimmunologiczne zaburzenia wielogruczołowe, Poliendokrynopatie autoimmunologiczne, Zespół Blizzarda, Zespół wielogruczołowy autoimmunologiczny typu II, Zespół wielogruczołowy autoimmunologiczny typu I, Zespół wielogruczołowy autoimmunologiczny typu III
Norwegian Autoimmune polyendokrinopatier, Polyendokrinopatier, autoimmune
Portuguese Poliendocrinopatias Autoimunes
Dutch auto-immuun; polyglandulair syndroom, deficiëntie; polyglandulair, auto-immuun, polyglandulair; deficiëntie, auto-immuun, polyglandulair; syndroom, auto-immuun, syndroom; auto-immuun polyglandulair, syndroom; polyglandulair, auto-immuun, Auto-immune polyglandulaire insufficiëntie, Auto-immune poly-endocrinopathie, Endocrinopathie auto-immune poly-, Poly-endocrinopathie, auto-immune
Italian Sindromi polighiandolari autoimmmuni
French Polyendocrinopathies auto-immunes, Polyendocrinopathies autoimmunes
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Autoimmune Syndrome Type II, Polyglandular (C0085860)

Concepts Disease or Syndrome (T047)
MSH D016884
ICD10 E31.0
SnomedCT 83728000, 190567009, 63805001
English Autoimmune Syndrome Type II, Polyglandular, Schmidt's Syndrome, Syndrome, Schmidt's, Diab mell,Add dis,myxoed, Polygland autoimm syn-typeII, Type 2 polyendoc autoimmun syn, POLYGLANDULAR AUTOIMMUNE SYNDROME, TYPE II, APS II, PGA II, DIABETES MELLITUS, ADDISON DISEASE, MYXEDEMA, POLYENDOCRINE AUTOIMMUNE SYNDROME, TYPE II, APS2, SCHMIDT SYNDROME, AUTOIMMUNE POLYENDOCRINE SYNDROME, TYPE II, Schmidt's syndrome (diagnosis), Carpenter syndrome, schmidt syndrome, schmidt's syndrome, schmidts syndrome, syndrome schmidt, Diabetes Mellitus, Addison's Disease, Myxedema, Polyglandular Deficiency Syndrome, Type 2, Autoimmune Polyendocrine Syndrome, Type 2, Multiple Endocrine Deficiency Syndrome, Type 2, Polyglandular Autoimmune Syndrome, Type 2, Diabetes Mellitus, Addison Disease, Myxedema, Autoimmune Polyendocrine Syndrome, Type II, Polyendocrine Autoimmune Syndrome, Type II, Syndrome, Schmidt, Schmidt Syndrome, Schmidt's syndrome (disorder), Addison's disease, toxic diffuse goiter AND insulin-dependent diabetes mellitus, Addison's disease, toxic diffuse goitre AND insulin-dependent diabetes mellitus, Schmidt's syndrome, APS type 2, Addison's disease with struma lymphomatosa, Polyglandular autoimmune syndrome, type 2, Diabetes mellitus, Addison's disease and myxedema, Diabetes mellitus, Addison's disease and myxoedema, PGA - Polyglandular autoimmune syndrome - type II, Polyglandular autoimmune syndrome - type II, Schmidt syndrome, Type 2 polyendocrine autoimmunity syndrome, Polyglandular autoimmune syndrome, type 2 (disorder), Primary hypothyroidism AND adrenocortical insufficiency, Schmidt, Addison's disease, toxic diffuse goiter and insulin-dependent diabetes mellitus, Primary hypothyroidism and adrenocortical insufficiency, Polyglandular Type II Autoimmune Syndrome, Polyglandular autoimmune syndrome type II, Autoimmune Polyglandular Syndrome Type II
Italian Sindrome autoimmune plurighiandolare tipo II
Japanese 多腺性自己免疫性症候群２型, ﾀｾﾝｾｲｼﾞｺﾒﾝｴｷｾｲｼｮｳｺｳｸﾞﾝ2ｶﾞﾀ
Czech Schmidtův syndrom (endokrinní), autoimunitní syndrom polyglandulární typ II, Polyglandulární autoimunitní syndrom typu II
French Polyendocrinopathie auto-immune de type 2, Polyendocrinopathie auto-immune de type II, Polyendocrinopathie autoimmune de type 2, Polyendocrinopathie autoimmune de type II, Syndrome de Schmidt
German Autoimmunsyndrom Typ II, polyglanduläres, polyglandulaeres Autoimmunsyndrom Typ II, Schmidt-Syndrom, Polyglanduläres Typ-II-Autoimmunsyndrom
Spanish síndrome autoinmune poliglandular, tipo 2 (trastorno), síndrome autoinmunitario poliglandular, tipo 2 (trastorno), enfermedad de Addison, bocio difuso tóxico Y diabetes mellitus insulino - dependiente, síndrome autoinmune poliglandular, tipo 2, síndrome autoinmunitario poliglandular, tipo 2, síndrome de Schmidt, enfermedad de Addison con estruma linfomatoso, síndrome de Schmidt (concepto no activo), hipotiroidismo primario Y enfermedad de Addison, enfermedad de Addison, bocio difuso tóxico Y diabetes mellitus insulinodependiente, hipotiroidismo primario e insuficiencia adrenocortical, APS tipo 2, enfermedad de Addison, bocio difuso tóxico y diabetes mellitus insulino - dependiente, Síndrome autoinmume poliglandular de tipo II, Síndrome de Schmidt, Síndrome Poliglandular Autoinmune Tipo II
Hungarian Polyglandularis autoimmun syndroma II. típus
Portuguese Síndrome Poliglandular Autoimune Tipo II, Síndrome auto-imune poliglandular tipo II, Síndrome de Schmidt
Dutch polyglandulair auto-immuun syndroom type II, Auto-immuun endocrien syndroom type II, poly-glandulair, Poly-glandulair endocrien syndroom type II, auto-immuun, Schmidt syndroom
Derived from the NIH UMLS (Unified Medical Language System)

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