Síndrome poliglandular autoinmune tipo 3: enfoque integral en el manejo de una enfermedad autoinmune multisistémica
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diciembre 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
Resumen
El Síndrome Poliglandular Autoinmune (SPA) tipo 3 es una enfermedad multisistémica caracterizada por la coexistencia de varias patologías autoinmunes, como tiroiditis de Hashimoto, vitíligo y enfermedad celíaca. Este síndrome afecta predominantemente a mujeres, con una fuerte predisposición genética asociada a haplotipos HLA. Su diagnóstico requiere la identificación de al menos dos enfermedades autoinmunes mediante historia clínica detallada, pruebas de laboratorio y criterios diagnósticos específicos. El abordaje terapéutico incluye intervenciones específicas para cada condición. La colaboración multidisciplinaria y la educación del paciente son esenciales para optimizar la calidad de vida, destacando la importancia del diagnóstico temprano y un enfoque personalizado.
Cómo citar
1.
Hernández JA, Therán JS, Gómez JA, Dulcey LA. Síndrome poliglandular autoinmune tipo 3: enfoque integral en el manejo de una enfermedad autoinmune multisistémica. 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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Detalles del artículo
Keywords
tiroiditis, vitíligo , abordaje terapéutico
References
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(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/
(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/
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Esta obra está bajo una licencia internacional Creative Commons Atribución 4.0.
Cómo citar
1.
Hernández JA, Therán JS, Gómez JA, Dulcey LA. Síndrome poliglandular autoinmune tipo 3: enfoque integral en el manejo de una enfermedad autoinmune multisistémica. 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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