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Autoimmune Polyglandular Syndrome

MedGen UID:
1383668
•Concept ID:
C4316913
•
Disease or Syndrome

Definition

A group of diverse conditions that are characterized by spontaneous, multi-organ autoimmunity, which target both endocrine (adrenal, gonad, pancreatic islet cells, parathyroid, pituitary, thyroid) and non-endocrine (gastrointestinal, integumentary, lymphatic) tissues. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAutoimmune Polyglandular Syndrome

Professional guidelines

PubMed

Autoimmune Primary Adrenal Insufficiency in Children.
Mourinho Bala N, Gonçalves RS, Serra Caetano J, Cardoso R, Dinis I, Mirante A
J Clin Res Pediatr Endocrinol 2022 Aug 25;14(3):308-312. Epub 2022 May 31 doi: 10.4274/jcrpe.galenos.2022.2021-11-9. PMID: 35633647Free PMC Article
Thyro-entero-gastric autoimmunity: Pathophysiology and implications for patient management.
Lahner E, Conti L, Cicone F, Capriello S, Cazzato M, Centanni M, Annibale B, Virili C
Best Pract Res Clin Endocrinol Metab 2020 Jan;34(1):101373. Epub 2019 Dec 11 doi: 10.1016/j.beem.2019.101373. PMID: 31864909
Anticytokine autoantibodies leading to infection: early recognition, diagnosis and treatment options.
Barcenas-Morales G, Cortes-Acevedo P, Doffinger R
Curr Opin Infect Dis 2019 Aug;32(4):330-336. doi: 10.1097/QCO.0000000000000561. PMID: 31116133Free PMC Article

Recent clinical studies

Etiology

Autoimmune amelogenesis imperfecta in patients with APS-1 and coeliac disease.
Gruper Y, Wolff ASB, Glanz L, Spoutil F, Marthinussen MC, Osickova A, Herzig Y, Goldfarb Y, Aranaz-Novaliches G, Dobeš J, Kadouri N, Ben-Nun O, Binyamin A, Lavi B, Givony T, Khalaila R, Gome T, Wald T, Mrazkova B, Sochen C, Besnard M, Ben-Dor S, Feldmesser E, Orlova EM, Hegedűs C, Lampé I, Papp T, Felszeghy S, Sedlacek R, Davidovich E, Tal N, Shouval DS, Shamir R, Guillonneau C, Szondy Z, Lundin KEA, Osicka R, Prochazka J, Husebye ES, Abramson J
Nature 2023 Dec;624(7992):653-662. Epub 2023 Nov 22 doi: 10.1038/s41586-023-06776-0. PMID: 37993717
Autoimmune polyglandular syndrome type 4: experience from a single reference center.
Gatta E, Anelli V, Cimino E, Di Lodovico E, Piovani E, Zammarchi I, Gozzoli G, Maltese V, Cavadini M, Agosti B, Delbarba A, Pirola I, Girelli A, Buoso C, Bambini F, Alfieri D, Bremi W, Facondo P, Lupo R, Bezzi F, Fredi M, Mazzola AM, Gandossi E, Saullo M, Marini F, Licini M, Pezzaioli LC, Pini L, Franceschini F, Ricci C, Cappelli C
Front Endocrinol (Lausanne) 2023;14:1236878. Epub 2023 Oct 23 doi: 10.3389/fendo.2023.1236878. PMID: 37937054Free PMC Article
Autoimmune Diseases in Patients with Premature Ovarian Insufficiency-Our Current State of Knowledge.
Szeliga A, Calik-Ksepka A, Maciejewska-Jeske M, Grymowicz M, Smolarczyk K, Kostrzak A, Smolarczyk R, Rudnicka E, Meczekalski B
Int J Mol Sci 2021 Mar 5;22(5) doi: 10.3390/ijms22052594. PMID: 33807517Free PMC Article
Autoimmune polyglandular syndromes.
Michels AW, Gottlieb PA
Nat Rev Endocrinol 2010 May;6(5):270-7. Epub 2010 Mar 23 doi: 10.1038/nrendo.2010.40. PMID: 20309000
Autoimmune hepatitis.
Obermayer-Straub P, Strassburg CP, Manns MP
J Hepatol 2000;32(1 Suppl):181-97. doi: 10.1016/s0168-8278(00)80425-0. PMID: 10728804

Diagnosis

Autoimmune amelogenesis imperfecta in patients with APS-1 and coeliac disease.
Gruper Y, Wolff ASB, Glanz L, Spoutil F, Marthinussen MC, Osickova A, Herzig Y, Goldfarb Y, Aranaz-Novaliches G, Dobeš J, Kadouri N, Ben-Nun O, Binyamin A, Lavi B, Givony T, Khalaila R, Gome T, Wald T, Mrazkova B, Sochen C, Besnard M, Ben-Dor S, Feldmesser E, Orlova EM, Hegedűs C, Lampé I, Papp T, Felszeghy S, Sedlacek R, Davidovich E, Tal N, Shouval DS, Shamir R, Guillonneau C, Szondy Z, Lundin KEA, Osicka R, Prochazka J, Husebye ES, Abramson J
Nature 2023 Dec;624(7992):653-662. Epub 2023 Nov 22 doi: 10.1038/s41586-023-06776-0. PMID: 37993717
Autoimmune polyglandular syndrome type 4: experience from a single reference center.
Gatta E, Anelli V, Cimino E, Di Lodovico E, Piovani E, Zammarchi I, Gozzoli G, Maltese V, Cavadini M, Agosti B, Delbarba A, Pirola I, Girelli A, Buoso C, Bambini F, Alfieri D, Bremi W, Facondo P, Lupo R, Bezzi F, Fredi M, Mazzola AM, Gandossi E, Saullo M, Marini F, Licini M, Pezzaioli LC, Pini L, Franceschini F, Ricci C, Cappelli C
Front Endocrinol (Lausanne) 2023;14:1236878. Epub 2023 Oct 23 doi: 10.3389/fendo.2023.1236878. PMID: 37937054Free PMC Article
A Review of Autoimmune Enteropathy and Its Associated Syndromes.
Chen CB, Tahboub F, Plesec T, Kay M, Radhakrishnan K
Dig Dis Sci 2020 Nov;65(11):3079-3090. Epub 2020 Aug 24 doi: 10.1007/s10620-020-06540-8. PMID: 32833153
The immunobiology and clinical features of type 1 autoimmune polyglandular syndrome (APS-1).
Guo CJ, Leung PSC, Zhang W, Ma X, Gershwin ME
Autoimmun Rev 2018 Jan;17(1):78-85. Epub 2017 Nov 4 doi: 10.1016/j.autrev.2017.11.012. PMID: 29108822
Autoimmune Addison's disease.
Betterle C, Morlin L
Endocr Dev 2011;20:161-172. Epub 2010 Dec 16 doi: 10.1159/000321239. PMID: 21164269

Therapy

Levothyroxine and insulin requirement in autoimmune polyglandular type 3 syndrome: a real-life study.
Guarnotta V, Pillitteri G, Gambino G, Radellini S, Vigneri E, Pizzolanti G, Giordano C
J Endocrinol Invest 2021 Jul;44(7):1387-1394. Epub 2020 Oct 24 doi: 10.1007/s40618-020-01421-3. PMID: 33099763Free PMC Article
Autoimmune Polyglandular Syndrome Type 2 and Pregnancy.
Yanachkova V, Chaveeva P, Staynova R, Milcheva R
Folia Med (Plovdiv) 2020 Jun 30;62(2):408-411. doi: 10.3897/folmed.62.e48580. PMID: 32666756
Autoimmune Addison's disease.
Betterle C, Morlin L
Endocr Dev 2011;20:161-172. Epub 2010 Dec 16 doi: 10.1159/000321239. PMID: 21164269
Autoimmune hepatitis.
Strassburg CP
Best Pract Res Clin Gastroenterol 2010 Oct;24(5):667-82. doi: 10.1016/j.bpg.2010.07.011. PMID: 20955969
Autoimmune polyglandular syndrome, type II.
Majeroni BA, Patel P
Am Fam Physician 2007 Mar 1;75(5):667-70. PMID: 17375512

Prognosis

Levothyroxine and insulin requirement in autoimmune polyglandular type 3 syndrome: a real-life study.
Guarnotta V, Pillitteri G, Gambino G, Radellini S, Vigneri E, Pizzolanti G, Giordano C
J Endocrinol Invest 2021 Jul;44(7):1387-1394. Epub 2020 Oct 24 doi: 10.1007/s40618-020-01421-3. PMID: 33099763Free PMC Article
The immunobiology and clinical features of type 1 autoimmune polyglandular syndrome (APS-1).
Guo CJ, Leung PSC, Zhang W, Ma X, Gershwin ME
Autoimmun Rev 2018 Jan;17(1):78-85. Epub 2017 Nov 4 doi: 10.1016/j.autrev.2017.11.012. PMID: 29108822
Immunogenetics of type 1 diabetes mellitus.
Morran MP, Vonberg A, Khadra A, Pietropaolo M
Mol Aspects Med 2015 Apr;42:42-60. Epub 2015 Jan 8 doi: 10.1016/j.mam.2014.12.004. PMID: 25579746Free PMC Article
Autoimmune hepatitis.
Strassburg CP
Best Pract Res Clin Gastroenterol 2010 Oct;24(5):667-82. doi: 10.1016/j.bpg.2010.07.011. PMID: 20955969
Autoimmune hepatitis.
Obermayer-Straub P, Strassburg CP, Manns MP
J Hepatol 2000;32(1 Suppl):181-97. doi: 10.1016/s0168-8278(00)80425-0. PMID: 10728804

Clinical prediction guides

Autoimmune polyglandular syndrome type 4: experience from a single reference center.
Gatta E, Anelli V, Cimino E, Di Lodovico E, Piovani E, Zammarchi I, Gozzoli G, Maltese V, Cavadini M, Agosti B, Delbarba A, Pirola I, Girelli A, Buoso C, Bambini F, Alfieri D, Bremi W, Facondo P, Lupo R, Bezzi F, Fredi M, Mazzola AM, Gandossi E, Saullo M, Marini F, Licini M, Pezzaioli LC, Pini L, Franceschini F, Ricci C, Cappelli C
Front Endocrinol (Lausanne) 2023;14:1236878. Epub 2023 Oct 23 doi: 10.3389/fendo.2023.1236878. PMID: 37937054Free PMC Article
Autoimmune Primary Adrenal Insufficiency in Children.
Mourinho Bala N, Gonçalves RS, Serra Caetano J, Cardoso R, Dinis I, Mirante A
J Clin Res Pediatr Endocrinol 2022 Aug 25;14(3):308-312. Epub 2022 May 31 doi: 10.4274/jcrpe.galenos.2022.2021-11-9. PMID: 35633647Free PMC Article
Immunogenetics of type 1 diabetes mellitus.
Morran MP, Vonberg A, Khadra A, Pietropaolo M
Mol Aspects Med 2015 Apr;42:42-60. Epub 2015 Jan 8 doi: 10.1016/j.mam.2014.12.004. PMID: 25579746Free PMC Article
The autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy or autoimmune polyglandular syndrome type 1.
Lankisch TO, Jaeckel E, Strassburg CP
Semin Liver Dis 2009 Aug;29(3):307-14. Epub 2009 Aug 12 doi: 10.1055/s-0029-1233535. PMID: 19676003
Autoimmune hepatitis. Part B: diagnosis.
Czaja AJ
Expert Rev Gastroenterol Hepatol 2007 Oct;1(1):129-43. doi: 10.1586/17474124.1.1.129. PMID: 19072441

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