PMID- 19382992 OWN - NLM STAT- MEDLINE DCOM- 20090522 LR - 20231213 IS - 1365-2796 (Electronic) IS - 0954-6820 (Linking) VI - 265 IP - 5 DP - 2009 May TI - Autoimmune polyendocrine syndrome type 1 (APS-1) as a model for understanding autoimmune polyendocrine syndrome type 2 (APS-2). PG - 530-40 LID - 10.1111/j.1365-2796.2009.02091.x [doi] AB - Autoimmune polyendocrine syndromes type 1 and 2 (APS-1 and APS-2) are diverse in regards to their component diseases and immunologic features of pathogenesis. Animal models and human studies highlight the importance of alleles of HLA (human leukocyte antigen)-like molecules determining tissue specific targeting that with the loss of tolerance leads to organ specific autoimmunity. Knowledge of the syndromes and component diseases allows clinicians to recognize and prevent illness prior to morbidity. With the current understanding of the syndromes, a paradigm for diagnosis, screening and treatment can be established. Once genetically susceptible individuals are identified screening for autoantibodies can be performed. Amongst autoantibody positive individuals, monitoring for physiologic decompensation, with a goal of treating prior to morbidity and in some cases mortality, follows. With continued basic and clinical research, therapies aimed at treating the underlying autoimmunity and disease prevention should become possible. FAU - Michels, A W AU - Michels AW AD - Department of Medicine, Barbara Davis Center for Childhood Diabetes, University of Colorado, Denver, Aurora, Colorado 80045, United States. Aaron.Michels@ucdenver.edu FAU - Eisenbarth, G S AU - Eisenbarth GS LA - eng GR - AI15416/AI/NIAID NIH HHS/United States GR - AI50964/AI/NIAID NIH HHS/United States GR - DK057538/DK/NIDDK NIH HHS/United States GR - DK32083/DK/NIDDK NIH HHS/United States GR - DK32493/DK/NIDDK NIH HHS/United States GR - MO1 RR00051/RR/NCRR NIH HHS/United States GR - MO1 RR00069/RR/NCRR NIH HHS/United States GR - P30 DK57516/DK/NIDDK NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Review PL - England TA - J Intern Med JT - Journal of internal medicine JID - 8904841 RN - 0 (Autoantibodies) RN - 0 (Histocompatibility Antigens Class II) RN - 0 (Transcription Factors) SB - IM MH - Adrenal Insufficiency/immunology MH - Animals MH - Autoantibodies/blood MH - Autoimmunity/genetics MH - Genetic Predisposition to Disease MH - Histocompatibility Antigens Class II/genetics MH - Humans MH - *Models, Immunological MH - Mutation MH - Polyendocrinopathies, Autoimmune/blood/diagnosis/*immunology MH - Self Tolerance/genetics MH - Syndrome MH - Transcription Factors/genetics MH - AIRE Protein RF - 75 EDAT- 2009/04/23 09:00 MHDA- 2009/05/23 09:00 CRDT- 2009/04/23 09:00 PHST- 2009/04/23 09:00 [entrez] PHST- 2009/04/23 09:00 [pubmed] PHST- 2009/05/23 09:00 [medline] AID - JIM2091 [pii] AID - 10.1111/j.1365-2796.2009.02091.x [doi] PST - ppublish SO - J Intern Med. 2009 May;265(5):530-40. doi: 10.1111/j.1365-2796.2009.02091.x.