Autoimmune polyendocrine syndrome type 3 explained

Autoimmune polyendocrine syndrome type 3
Synonyms:	Autoimmune polyendocrinopathy type 3, Autoimmune polyglandular syndrome type 3, APS type 3, APS3, PAS3.^[1]
Field:	Endocrinology, Rheumatology
Symptoms:	Any symptoms associated with its constituent diseases
Complications:	Autoimmune thyroiditis (Always), Celiac Disease, Type I Diabetes, Autoimmune hypophysitis, Systemic Lupus Erythematosus, Sjögren's Syndrome, Vitiligo (May or May not be Present)
Onset:	Any Age
Duration:	Lifelong
Types:	APS 3A: Autoimmune thyroiditis with Immune Mediated diabetes mellitus;APS 3B: Autoimmune thyroiditis with Pernicious anemia;APS 3C: Autoimmune thyroiditis with Vitiligo and/or Alopecia and/or Another Organ Specific Disease
Causes:	Combination of Genetic and Environmental Factors
Risks:	Family History of APS III or other Autoimmune Diseases
Diagnosis:	Serum antibody assays; Autoimmune Thyroiditis Required for Diagnosis
Differential:	Autoimmune Polyendocrine Syndrome Type II, APECED, IPEX
Prevention:	N/A
Treatment:	Depends on Constituent Diseases
Prognosis:	Normal Lifespan
Frequency:	~ 2-3% of population

Autoimmune polyendocrine syndrome, type 3 is a condition characterized by the coexistence of autoimmune thyroiditis and at least one other autoimmune disease (excluding Addison's Disease).^[2] Based on other organ-specific autoimmune involvement, there are multiple subtypes that are classified: type 3a shows thyroid autoimmune disease in conjunction with type 1 diabetes, type 3b shows thyroid autoimmune disease in conjunction with pernicious anemia (PA), and type 3c shows thyroid autoimmune disease in conjunction with alopecia, vitiligo, or other organ-specific autoimmune disease.^[3]

Related Conditions

The hallmark of autoimmune polyglandular syndromes (APS) is the existence of autoimmune reactions directed against multiple endocrine and non-endocrine organs. There have been described as four primary types:^[4]

• APS-1 [Autoimmune-Polyendocrine-Candidiasis-Ectodermal-Dystrophy Syndrome (APECED)]: Chronic hypoparathyroidism, autoimmune Addison's disease, and chronic candidiasis (two conditions must exist for the term to be used).^[4]
• APS-2 (Schmidt's disease): Autoimmune Addison's disease (constantly present), in conjunction with either type 1 diabetes mellitus (DM) or autoimmune thyroid disease.^[4]
• APS-3: Type 1 diabetes, atrophic gastritis, pernicious anemia, vitiligo, alopecia, and myasthenia gravis, and autoimmune thyroid disease; Addison's disease and/or hypoparathyroidism are not included in this association.^[4]
• APS-4: Combinations that weren't in the earlier groups.^[4]

Signs and symptoms

Most patients with autoimmune polyendocrine syndrome type 3 have autoimmune thyroid diseases associated with only one other autoimmune disease; these associations are most frequently with either type 1 diabetes (20–30% of cases)^[5] or chronic atrophic gastritis (39 percent of cases).^[6] Other disorders associated with autoimmune polyendocrine syndrome type 3 are pernicious anemia, vitiligo, alopecia, and myasthenia gravis.^[4]

Epidemiology

Autoimmune polyendocrine syndrome, type 3 has a frequency of 1.4 to 2 per 100,000 people, with no discernible ethnic group preference.^[7]

Further reading

• 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 . Journal of Endocrinological Investigation . 46 . 4 . 2023-01-07 . 1720-8386 . 10.1007/s40618-022-01994-1 . 643–665 . 36609775 . 255501278 . none.
• Horie . Ichiro . Kawasaki . Eiji . Ando . Takao . Kuwahara . Hironaga . Abiru . Norio . Usa . Toshiro . Yamasaki . Hironori . Ejima . Eri . Kawakami . Atsushi . Clinical and Genetic Characteristics of Autoimmune Polyglandular Syndrome Type 3 Variant in the Japanese Population . The Journal of Clinical Endocrinology & Metabolism . The Endocrine Society . 97 . 6 . June 1, 2012 . 0021-972X . 10.1210/jc.2011-3109 . E1043–E1050 . 22466347 . none. 10069/29379 . free .

Notes and References

1. Web site: Monarch Initiative . Monarch Initiative . January 27, 2024.
2. Betterle . Corrado . Garelli . Silvia . Coco . Graziella . Burra . Patrizia . A rare combination of type 3 autoimmune polyendocrine syndrome (APS-3) or multiple autoimmune syndrome (MAS-3) . Autoimmunity Highlights . Springer Science and Business Media LLC . 5 . 1 . February 11, 2014 . 2038-0305 . 10.1007/s13317-013-0055-6 . 27–31. 26000153 . 4389012 .
3. Apolinario . Michael . Brussels . Aaron . Cook . Curtiss B. . Yang . Shaun . Autoimmune polyglandular syndrome type 3: A case report of an unusual presentation and literature review . Clinical Case Reports . 10 . 2 . 2022 . e05391 . 2050-0904 . 35140971 . 8815091 . 10.1002/ccr3.5391 .
4. Quintos . Jb . Grover . Monica . Boney . Charlotte M . Salas . Max . Autoimmune polyglandular syndrome Type 3 and growth hormone deficiency . Pediatric Diabetes . 11 . 6 . 2010 . 10.1111/j.1399-5448.2009.00622.x . 438–442. 8964242 .
5. Warncke . Katharina . Fröhlich-Reiterer . Elke E. . Thon . Angelika . Hofer . Sabine E. . Wiemann . Dagobert . Holl . Reinhard W. . Polyendocrinopathy in Children, Adolescents, and Young Adults With Type 1 Diabetes . Diabetes Care . American Diabetes Association . 33 . 9 . June 14, 2010 . 0149-5992 . 10.2337/dc10-0404 . 2010–2012. 20551013 . 2928352 .
6. Lahner . Edith . Centanni . Marco . Agnello . Giacoma . Gargano . Lucilla . Vannella . Lucy . Iannoni . Carlo . Delle Fave . Gianfranco . Annibale . Bruno . Occurrence and Risk Factors for Autoimmune Thyroid Disease in Patients with Atrophic Body Gastritis . The American Journal of Medicine . Elsevier BV . 121 . 2 . 2008 . 0002-9343 . 10.1016/j.amjmed.2007.09.025 . 136–141. 18261502 .
7. Frommer . Lara . Kahaly . George J . Autoimmune Polyendocrinopathy . The Journal of Clinical Endocrinology & Metabolism . The Endocrine Society . 104 . 10 . April 26, 2019 . 0021-972X . 10.1210/jc.2019-00602 . 4769–4782. 31127843 .