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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

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
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
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

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
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
Van den Driessche A, Eenkhoorn V, Van Gaal L, De Block C
Neth J Med 2009 Dec;67(11):376-87. PMID: 20009114
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
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

Garelli S, Dalla Costa M, Sabbadin C, Barollo S, Rubin B, Scarpa R, Masiero S, Fierabracci A, Bizzarri C, Crinò A, Cappa M, Valenzise M, Meloni A, De Bellis AM, Giordano C, Presotto F, Perniola R, Capalbo D, Salerno MC, Stigliano A, Radetti G, Camozzi V, Greggio NA, Bogazzi F, Chiodini I, Pagotto U, Black SK, Chen S, Rees Smith B, Furmaniak J, Weber G, Pigliaru F, De Sanctis L, Scaroni C, Betterle C
J Endocrinol Invest 2021 Nov;44(11):2493-2510. Epub 2021 May 18 doi: 10.1007/s40618-021-01585-6. PMID: 34003463Free PMC Article
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
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
Strassburg CP
Best Pract Res Clin Gastroenterol 2010 Oct;24(5):667-82. doi: 10.1016/j.bpg.2010.07.011. PMID: 20955969
Van den Driessche A, Eenkhoorn V, Van Gaal L, De Block C
Neth J Med 2009 Dec;67(11):376-87. PMID: 20009114

Therapy

Atkinson M, Lansdown AJ
Best Pract Res Clin Endocrinol Metab 2022 May;36(3):101635. Epub 2022 Feb 28 doi: 10.1016/j.beem.2022.101635. PMID: 35382989
Betterle C, Morlin L
Endocr Dev 2011;20:161-172. Epub 2010 Dec 16 doi: 10.1159/000321239. PMID: 21164269
Strassburg CP
Best Pract Res Clin Gastroenterol 2010 Oct;24(5):667-82. doi: 10.1016/j.bpg.2010.07.011. PMID: 20955969
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
Majeroni BA, Patel P
Am Fam Physician 2007 Mar 1;75(5):667-70. PMID: 17375512

Prognosis

Garelli S, Dalla Costa M, Sabbadin C, Barollo S, Rubin B, Scarpa R, Masiero S, Fierabracci A, Bizzarri C, Crinò A, Cappa M, Valenzise M, Meloni A, De Bellis AM, Giordano C, Presotto F, Perniola R, Capalbo D, Salerno MC, Stigliano A, Radetti G, Camozzi V, Greggio NA, Bogazzi F, Chiodini I, Pagotto U, Black SK, Chen S, Rees Smith B, Furmaniak J, Weber G, Pigliaru F, De Sanctis L, Scaroni C, Betterle C
J Endocrinol Invest 2021 Nov;44(11):2493-2510. Epub 2021 May 18 doi: 10.1007/s40618-021-01585-6. PMID: 34003463Free PMC Article
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
Strassburg CP
Best Pract Res Clin Gastroenterol 2010 Oct;24(5):667-82. doi: 10.1016/j.bpg.2010.07.011. PMID: 20955969
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
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

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
Szlendak-Sauer K, Jakubik D, Kunicki M, Skórska J, Smolarczyk R
Eur J Obstet Gynecol Reprod Biol 2016 Aug;203:61-5. Epub 2016 May 20 doi: 10.1016/j.ejogrb.2016.05.023. PMID: 27240263
Horie I, Kawasaki E, Ando T, Kuwahara H, Abiru N, Usa T, Yamasaki H, Ejima E, Kawakami A
J Clin Endocrinol Metab 2012 Jun;97(6):E1043-50. Epub 2012 Mar 30 doi: 10.1210/jc.2011-3109. PMID: 22466347
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
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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