Autoimmune polyglandular syndrome type 3: comprehensive approach in the management of a multisystem autoimmune disease.
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Published
December 30, 2025
Jorge Andrés Hernández
Juan Sebastián Therán
Jaime Alberto Gómez
Luis Andrés Dulcey
https://orcid.org/0009-0001-5758-5965
Juan Sebastián Therán
Jaime Alberto Gómez
Luis Andrés Dulcey
Abstract
Autoimmune Polyendocrine Syndrome (APS) type 3 is a multisystem disorder characterized by the coexistence of multiple autoimmune conditions, including Hashimoto's thyroiditis, vitiligo, and celiac disease. This syndrome predominantly affects women, with a strong genetic predisposition associated with HLA haplotypes. Diagnosis requires the identification of at least two concurrent autoimmune diseases through detailed clinical history, laboratory testing, and specific diagnostic criteria. The therapeutic approach involves targeted interventions for each condition. Multidisciplinary collaboration and patient education are essential to improving quality of life, emphasizing the importance of early diagnosis and a personalized management strategy.
How to Cite
1.
Hernández JA, Therán JS, Gómez JA, Dulcey LA. Autoimmune polyglandular syndrome type 3: comprehensive approach in the management of a multisystem autoimmune disease. Rev. parag. reumatol. [Internet]. 2025 Dec. 30 [cited 2026 Sep. 22];11(2):87-90. Available from: https://revista.spr.org.py/index.php/spr/article/view/226
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Keywords
thyroiditis , vitiligo , therapeutic approach
References
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(3) Jiang Q, Wu T, Zhang Y, Wang S, Wang L, Su W, et al. Case Report: A Rare Case of Coexisting of Autoimmune Polyglandular Syndrome Type 3 and Isolated Gonadotropin-Releasing Hormone Deficiency. Front Immunol [Internet]. 2021 Sep 14 [cited 2025 Jan 17];12. Available from: https://pubmed.ncbi.nlm.nih.gov/34594339/
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(5) Capo A, Amerio P. Polyglandular autoimmune syndrome type III with a prevalence of cutaneous features. Clin Exp Dermatol [Internet]. 2017 Jan 1 [cited 2025 Jan 17];42(1):61–3. Available from: https://pubmed.ncbi.nlm.nih.gov/27859474/
(6) Betterle C, Furmaniak J, Sabbadin C, Scaroni C, Presotto F. Type 3 autoimmune polyglandular syndrome (APS-3) or type 3 multiple autoimmune syndrome (MAS-3): an expanding galaxy. J Endocrinol Invest [Internet]. 2023 Apr 1 [cited 2025 Jan 17];46(4):643–65. Available from: https://pubmed.ncbi.nlm.nih.gov/36609775/
(7) Dittmar M, Kahaly GJ. Genetics of the autoimmune polyglandular syndrome type 3 variant. Thyroid [Internet]. 2010 [cited 2025 Jan 17];20(7):737–43. Available from: https://pubmed.ncbi.nlm.nih.gov/20578896/
(8) Vita R, Santaguida MG, Virili C, Segni M, Galletti M, Mandolfino M, et al. Serum Thyroid Hormone Antibodies Are Frequent in Patients with Polyglandular Autoimmune Syndrome Type 3, Particularly in Those Who Require Thyroxine Treatment. Front Endocrinol (Lausanne) [Internet]. 2017 Aug 28 [cited 2025 Jan 17];8(AUG). Available from: https://pubmed.ncbi.nlm.nih.gov/28894436/
(9) De Marchi SU, Cecchin E, De Marchi S. Autoimmune spontaneous chronic urticaria and generalized myasthenia gravis in a patient with polyglandular autoimmune syndrome type 3. Muscle Nerve [Internet]. 2015 Sep 1 [cited 2025 Jan 17];52(3):440–4. Available from: https://pubmed.ncbi.nlm.nih.gov/25808274/
(10) Horie I, Kawasaki E, Ando T, Kuwahara H, Abiru N, Usa T, et al. Clinical and genetic characteristics of autoimmune polyglandular syndrome type 3 variant in the Japanese population. J Clin Endocrinol Metab [Internet]. 2012 [cited 2025 Jan 17];97(6). Available from: https://pubmed.ncbi.nlm.nih.gov/22466347/
(11) Hadwen TI, Foster K, Buchanan J, Sutcliffe S, Sinha AK. A case of nonischemic cardiomyopathy associated with autoimmune polyglandular syndrome type III. Endocr Pract [Internet]. 2014 Oct 1 [cited 2025 Jan 17];20(10):e183–6. Available from: https://pubmed.ncbi.nlm.nih.gov/24936552/
(12) Li CW, Osman R, Menconi F, Concepcion ES, Tomer Y. Flexible peptide recognition by HLA-DR triggers specific autoimmune T-cell responses in autoimmune thyroiditis and diabetes. J Autoimmun [Internet]. 2017 Jan 1 [cited 2025 Jan 17];76:1–9. Available from: https://pubmed.ncbi.nlm.nih.gov/27670087/
(2) Tian S, Xu B, Liu Z, Liu R. Autoimmune polyglandular syndrome type III associated with antineutrophil cytoplasmic autoantibody-mediated crescentic glomerulonephritis: A case report and literature review. Medicine [Internet]. 2020 [cited 2025 Jan 17];99(7). Available from: https://pubmed.ncbi.nlm.nih.gov/32049851/
(3) Jiang Q, Wu T, Zhang Y, Wang S, Wang L, Su W, et al. Case Report: A Rare Case of Coexisting of Autoimmune Polyglandular Syndrome Type 3 and Isolated Gonadotropin-Releasing Hormone Deficiency. Front Immunol [Internet]. 2021 Sep 14 [cited 2025 Jan 17];12. Available from: https://pubmed.ncbi.nlm.nih.gov/34594339/
(4) Fernández Miró M, Colom Comí C, Godoy Lorenzo R. Autoinmune polyendocrinopathy. Med Clin (Barc) [Internet]. 2021 Sep 10 [cited 2025 Jan 17];157(5):241–6. Available from: https://pubmed.ncbi.nlm.nih.gov/33958142/
(5) Capo A, Amerio P. Polyglandular autoimmune syndrome type III with a prevalence of cutaneous features. Clin Exp Dermatol [Internet]. 2017 Jan 1 [cited 2025 Jan 17];42(1):61–3. Available from: https://pubmed.ncbi.nlm.nih.gov/27859474/
(6) Betterle C, Furmaniak J, Sabbadin C, Scaroni C, Presotto F. Type 3 autoimmune polyglandular syndrome (APS-3) or type 3 multiple autoimmune syndrome (MAS-3): an expanding galaxy. J Endocrinol Invest [Internet]. 2023 Apr 1 [cited 2025 Jan 17];46(4):643–65. Available from: https://pubmed.ncbi.nlm.nih.gov/36609775/
(7) Dittmar M, Kahaly GJ. Genetics of the autoimmune polyglandular syndrome type 3 variant. Thyroid [Internet]. 2010 [cited 2025 Jan 17];20(7):737–43. Available from: https://pubmed.ncbi.nlm.nih.gov/20578896/
(8) Vita R, Santaguida MG, Virili C, Segni M, Galletti M, Mandolfino M, et al. Serum Thyroid Hormone Antibodies Are Frequent in Patients with Polyglandular Autoimmune Syndrome Type 3, Particularly in Those Who Require Thyroxine Treatment. Front Endocrinol (Lausanne) [Internet]. 2017 Aug 28 [cited 2025 Jan 17];8(AUG). Available from: https://pubmed.ncbi.nlm.nih.gov/28894436/
(9) De Marchi SU, Cecchin E, De Marchi S. Autoimmune spontaneous chronic urticaria and generalized myasthenia gravis in a patient with polyglandular autoimmune syndrome type 3. Muscle Nerve [Internet]. 2015 Sep 1 [cited 2025 Jan 17];52(3):440–4. Available from: https://pubmed.ncbi.nlm.nih.gov/25808274/
(10) Horie I, Kawasaki E, Ando T, Kuwahara H, Abiru N, Usa T, et al. Clinical and genetic characteristics of autoimmune polyglandular syndrome type 3 variant in the Japanese population. J Clin Endocrinol Metab [Internet]. 2012 [cited 2025 Jan 17];97(6). Available from: https://pubmed.ncbi.nlm.nih.gov/22466347/
(11) Hadwen TI, Foster K, Buchanan J, Sutcliffe S, Sinha AK. A case of nonischemic cardiomyopathy associated with autoimmune polyglandular syndrome type III. Endocr Pract [Internet]. 2014 Oct 1 [cited 2025 Jan 17];20(10):e183–6. Available from: https://pubmed.ncbi.nlm.nih.gov/24936552/
(12) Li CW, Osman R, Menconi F, Concepcion ES, Tomer Y. Flexible peptide recognition by HLA-DR triggers specific autoimmune T-cell responses in autoimmune thyroiditis and diabetes. J Autoimmun [Internet]. 2017 Jan 1 [cited 2025 Jan 17];76:1–9. Available from: https://pubmed.ncbi.nlm.nih.gov/27670087/
Section
CASE REPORTS
How to Cite
1.
Hernández JA, Therán JS, Gómez JA, Dulcey LA. Autoimmune polyglandular syndrome type 3: comprehensive approach in the management of a multisystem autoimmune disease. Rev. parag. reumatol. [Internet]. 2025 Dec. 30 [cited 2026 Sep. 22];11(2):87-90. Available from: https://revista.spr.org.py/index.php/spr/article/view/226
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