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Autoimmune polyendocrinopathy type 3

MedGen UID:
453060
Concept ID:
C1535942
Disease or Syndrome
Synonyms: Autoimmune Polyglandular Syndrome Type III; Autoimmune Polyglandular Syndrome, Type 3; Autoimmune Syndrome Type III, Polyglandular; Polyglandular Autoimmune Syndrome, Type 3; Polyglandular Type III Autoimmune Syndrome
SNOMED CT: Autoimmune polyendocrine syndrome type 3 (449731009)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
 
Monarch Initiative: MONDO:0016422
Orphanet: ORPHA227982

Definition

A rare, endocrine disease characterized by autoimmune thyroid disease associated with at least one other autoimmune disease, such as type I diabetes mellitus, chronic atrophic gastritis, pernicious anemia, vitiligo, alopecia, or myasthenia gravis, but excluding Addison disease. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAutoimmune polyendocrinopathy type 3

Professional guidelines

PubMed

Ben-Skowronek I
Genes (Basel) 2021 Feb 24;12(3) doi: 10.3390/genes12030323. PMID: 33668198Free PMC Article
Kirou KA, Gkrouzman E
Clin Immunol 2013 Sep;148(3):303-12. Epub 2013 Mar 1 doi: 10.1016/j.clim.2013.02.013. PMID: 23566912
Capalbo D, Mazza C, Giordano R, Improda N, Arvat E, Cervato S, Morlin L, Pignata C, Betterle C, Salerno M
J Endocrinol Invest 2012 Feb;35(2):169-73. Epub 2011 Apr 20 doi: 10.3275/7677. PMID: 21508664

Recent clinical studies

Etiology

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
Le Voyer T, Parent AV, Liu X, Cederholm A, Gervais A, Rosain J, Nguyen T, Perez Lorenzo M, Rackaityte E, Rinchai D, Zhang P, Bizien L, Hancioglu G, Ghillani-Dalbin P, Charuel JL, Philippot Q, Gueye MS, Maglorius Renkilaraj MRL, Ogishi M, Soudée C, Migaud M, Rozenberg F, Momenilandi M, Riller Q, Imberti L, Delmonte OM, Müller G, Keller B, Orrego J, Franco Gallego WA, Rubin T, Emiroglu M, Parvaneh N, Eriksson D, Aranda-Guillen M, Berrios DI, Vong L, Katelaris CH, Mustillo P, Raedler J, Bohlen J, Bengi Celik J, Astudillo C, Winter S; NF-κB Consortium; COVID Human Genetic Effort, McLean C, Guffroy A, DeRisi JL, Yu D, Miller C, Feng Y, Guichard A, Béziat V, Bustamante J, Pan-Hammarström Q, Zhang Y, Rosen LB, Holland SM, Bosticardo M, Kenney H, Castagnoli R, Slade CA, Boztuğ K, Mahlaoui N, Latour S, Abraham RS, Lougaris V, Hauck F, Sediva A, Atschekzei F, Sogkas G, Poli MC, Slatter MA, Palterer B, Keller MD, Pinzon-Charry A, Sullivan A, Droney L, Suan D, Wong M, Kane A, Hu H, Ma C, Grombiříková H, Ciznar P, Dalal I, Aladjidi N, Hie M, Lazaro E, Franco J, Keles S, Malphettes M, Pasquet M, Maccari ME, Meinhardt A, Ikinciogullari A, Shahrooei M, Celmeli F, Frosk P, Goodnow CC, Gray PE, Belot A, Kuehn HS, Rosenzweig SD, Miyara M, Licciardi F, Servettaz A, Barlogis V, Le Guenno G, Herrmann VM, Kuijpers T, Ducoux G, Sarrot-Reynauld F, Schuetz C, Cunningham-Rundles C, Rieux-Laucat F, Tangye SG, Sobacchi C, Doffinger R, Warnatz K, Grimbacher B, Fieschi C, Berteloot L, Bryant VL, Trouillet Assant S, Su H, Neven B, Abel L, Zhang Q, Boisson B, Cobat A, Jouanguy E, Kampe O, Bastard P, Roifman CM, Landegren N, Notarangelo LD, Anderson MS, Casanova JL, Puel A
Nature 2023 Nov;623(7988):803-813. Epub 2023 Nov 8 doi: 10.1038/s41586-023-06717-x. PMID: 37938781Free PMC Article
Fernández Miró M, Colom Comí C, Godoy Lorenzo R
Med Clin (Barc) 2021 Sep 10;157(5):241-246. Epub 2021 May 3 doi: 10.1016/j.medcli.2021.02.004. PMID: 33958142
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
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

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
Betterle C, Furmaniak J, Sabbadin C, Scaroni C, Presotto F
J Endocrinol Invest 2023 Apr;46(4):643-665. Epub 2023 Jan 7 doi: 10.1007/s40618-022-01994-1. PMID: 36609775
Fernández Miró M, Colom Comí C, Godoy Lorenzo R
Med Clin (Barc) 2021 Sep 10;157(5):241-246. Epub 2021 May 3 doi: 10.1016/j.medcli.2021.02.004. PMID: 33958142
Ben-Skowronek I
Genes (Basel) 2021 Feb 24;12(3) doi: 10.3390/genes12030323. PMID: 33668198Free PMC Article
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

Therapy

De Marchi G, de Pretis N, Gabrieletto EM, Amodio A, Davì V, Crinò SF, Gabbrielli A, Ciccocioppo R, Frulloni L
Pancreas 2021 Nov-Dec 01;50(10):1363-1367. doi: 10.1097/MPA.0000000000001925. PMID: 35041334
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
Horino T, Ogasawara M, Ichii O, Terada Y
Rom J Intern Med 2020 Mar 1;58(1):40-43. doi: 10.2478/rjim-2019-0024. PMID: 31756165
Kirou KA, Gkrouzman E
Clin Immunol 2013 Sep;148(3):303-12. Epub 2013 Mar 1 doi: 10.1016/j.clim.2013.02.013. PMID: 23566912
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

Prognosis

Fernández Miró M, Colom Comí C, Godoy Lorenzo R
Med Clin (Barc) 2021 Sep 10;157(5):241-246. Epub 2021 May 3 doi: 10.1016/j.medcli.2021.02.004. PMID: 33958142
Ben-Skowronek I
Genes (Basel) 2021 Feb 24;12(3) doi: 10.3390/genes12030323. PMID: 33668198Free PMC Article
Bin Dhuban K, Piccirillo CA
Curr Opin Allergy Clin Immunol 2015 Dec;15(6):525-32. doi: 10.1097/ACI.0000000000000214. PMID: 26485097
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

Rackaityte E, Proekt I, Miller HS, Ramesh A, Brooks JF, Kung AF, Mandel-Brehm C, Yu D, Zamecnik CR, Bair R, Vazquez SE, Sunshine S, Abram CL, Lowell CA, Rizzuto G, Wilson MR, Zikherman J, Anderson MS, DeRisi JL
JCI Insight 2023 Dec 8;8(23) doi: 10.1172/jci.insight.174976. PMID: 37934865Free PMC Article
Fernández Miró M, Colom Comí C, Godoy Lorenzo R
Med Clin (Barc) 2021 Sep 10;157(5):241-246. Epub 2021 May 3 doi: 10.1016/j.medcli.2021.02.004. PMID: 33958142
Bin Dhuban K, Piccirillo CA
Curr Opin Allergy Clin Immunol 2015 Dec;15(6):525-32. doi: 10.1097/ACI.0000000000000214. PMID: 26485097
Couturier A, Saugier-Veber P, Carel JC, Bertherat J, Brézin AP
Cornea 2015 Sep;34(9):1086-91. doi: 10.1097/ICO.0000000000000513. PMID: 26114819
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

Recent systematic reviews

Hou AN, Wang Y, Pan YQ
J Clin Immunol 2023 Jul;43(5):979-988. Epub 2023 Mar 3 doi: 10.1007/s10875-023-01456-0. PMID: 36867340
Zhao Z, Wang X, Bao XQ, Ning J, Shang M, Zhang D
Cancer Immunol Immunother 2021 Jun;70(6):1527-1540. Epub 2020 Nov 16 doi: 10.1007/s00262-020-02699-1. PMID: 33200250Free PMC Article
Pham-Dobor G, Hanák L, Hegyi P, Márta K, Párniczky A, Gergics M, Sarlós P, Erőss B, Mezősi E
J Endocrinol Invest 2020 Sep;43(9):1-9. Epub 2020 Aug 17 doi: 10.1007/s40618-020-01229-1. PMID: 32227311Free PMC Article

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