{"term":{"id":"12923","title":"autoimmune polyendocrine syndrome type 1 or 2","longtitle":"IUPAC Gold Book - autoimmune polyendocrine syndrome type 1 or 2","doi":"10.1351\/goldbook.12923","code":"12923","status":"current","initialism":"<em>initialism<\/em>: APS","definitions":[{"id":1,"text":"Heterogeneous group of rare diseases characterized by autoimmune activity against more than one endocrine organ, although non-endocrine organs can also be affected.","notes":{"1":"Autoimmune polyendocrine syndrome, type 1 is known as the candidiasis-hypoparathyroidism-Addison disease syndrome after its main features: A mild immune deficiency, leading to persistent mucosal and cutaneous infections with Candida yeasts. There is also decreased function of the spleen (asplenism).Autoimmune dysfunction of the parathyroid gland (leading to hypocalcemia) and the adrenal gland (Addison disease).","2":"Autoimmune polyendocrine syndrome, type 2 (also known as Schmidt syndrome) is more heterogeneous, occurs more often and has not been linked to one gene. Features of this syndrome are Addison disease, hypothyroidism (Hashimoto thyroiditis), and diabetes mellitus type 1. Patients are at a higher risk when they carry a particular human leukocyte antigen (HLA) genotype (e.g., DQ2, DQ8, and DRB1*0404)."},"links":[{"term":"Addison disease","url":"https:\/\/goldbook.iupac.org\/terms\/view\/12845"},{"term":"Hashimoto thyroiditis","url":"https:\/\/goldbook.iupac.org\/terms\/view\/13195"},{"term":"autoimmune","url":"https:\/\/goldbook.iupac.org\/terms\/view\/12918"},{"term":"candidiasis","url":"https:\/\/goldbook.iupac.org\/terms\/view\/12986"},{"term":"diabetes mellitus type 1","url":"https:\/\/goldbook.iupac.org\/terms\/view\/13092"},{"term":"human leukocyte antigen (HLA)","url":"https:\/\/goldbook.iupac.org\/terms\/view\/13230"},{"term":"mucosal","url":"https:\/\/goldbook.iupac.org\/terms\/view\/13473"},{"term":"spleen","url":"https:\/\/goldbook.iupac.org\/terms\/view\/13690"}],"sources":["PAC, 2012, 84, 1113. 'IUPAC glossary of terms used in immunotoxicology (IUPAC Recommendations 2012)' on page 1130 (https:\/\/doi.org\/10.1351\/PAC-REC-11-06-03)"]}],"altoutputs":{"html":"https:\/\/goldbook.iupac.org\/terms\/view\/12923\/html","xml":"https:\/\/goldbook.iupac.org\/terms\/view\/12923\/xml","plain":"https:\/\/goldbook.iupac.org\/terms\/view\/12923\/plain"},"citation":"Citation: 'autoimmune polyendocrine syndrome type 1 or 2' in IUPAC Compendium of Chemical Terminology, 5th ed. International Union of Pure and Applied Chemistry; 2025. Online version 5.0.0, 2025. 10.1351\/goldbook.12923","license":"The IUPAC Gold Book is licensed under Creative Commons Attribution-ShareAlike CC BY-SA 4.0 International (https:\/\/creativecommons.org\/licenses\/by-sa\/4.0\/) for individual terms.","collection":"If you are interested in licensing the Gold Book for commercial use, please contact the IUPAC Executive Director at executivedirector@iupac.org .","disclaimer":"The International Union of Pure and Applied Chemistry (IUPAC) is continuously reviewing and, where needed, updating terms in the Compendium of Chemical Terminology (the IUPAC Gold Book). Users of these terms are encouraged to include the version of a term with its use and to check regularly for updates to term definitions that you are using.","accessed":"2026-07-01T07:34:57+00:00"}}