Medicine:Autoimmune polyendocrine syndrome type 3

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Autoimmune polyendocrine syndrome type 3
Other namesAutoimmune polyendocrinopathy type 3, Autoimmune polyglandular syndrome type 3, APS type 3, APS3, PAS3.[1]
SpecialtyEndocrinology, Rheumatology
SymptomsAny symptoms associated with its constituent diseases
ComplicationsAutoimmune thyroiditis (Always), Celiac Disease, Type I Diabetes, Autoimmune hypophysitis, Systemic Lupus Erythematosus, Sjögren's Syndrome, Vitiligo (May or May not be Present)
Usual onsetAny Age
DurationLifelong
TypesAPS 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
CausesCombination of Genetic and Environmental Factors
Risk factorsFamily History of APS III or other Autoimmune Diseases
Diagnostic methodSerum antibody assays; Autoimmune Thyroiditis Required for Diagnosis
Differential diagnosisAutoimmune Polyendocrine Syndrome Type II, APECED, IPEX
PreventionN/A
TreatmentDepends on Constituent Diseases
PrognosisNormal Lifespan
Frequency~ 2-3% of population

Autoimmune polyendocrine syndrome, type 3 is characterized by the coexistence of two autoimmune illnesses, not including Addison's disease, and an autoimmune thyroid 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]

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]

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]

References

  1. ↑ "Monarch Initiative". https://monarchinitiative.org/MONDO:0016422. 
  2. ↑ Betterle, Corrado; Garelli, Silvia; Coco, Graziella; Burra, Patrizia (February 11, 2014). "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): 27–31. doi:10.1007/s13317-013-0055-6. ISSN 2038-0305. PMID 26000153. 
  3. ↑ Apolinario, Michael; Brussels, Aaron; Cook, Curtiss B.; Yang, Shaun (2022). "Autoimmune polyglandular syndrome type 3: A case report of an unusual presentation and literature review". Clinical Case Reports 10 (2): e05391. doi:10.1002/ccr3.5391. ISSN 2050-0904. PMID 35140971. 
  4. ↑ 4.0 4.1 4.2 4.3 4.4 4.5 Quintos, Jb; Grover, Monica; Boney, Charlotte M; Salas, Max (2010). "Autoimmune polyglandular syndrome Type 3 and growth hormone deficiency". Pediatric Diabetes 11 (6): 438–442. doi:10.1111/j.1399-5448.2009.00622.x. 
  5. ↑ Warncke, Katharina; Fröhlich-Reiterer, Elke E.; Thon, Angelika; Hofer, Sabine E.; Wiemann, Dagobert; Holl, Reinhard W. (June 14, 2010). "Polyendocrinopathy in Children, Adolescents, and Young Adults With Type 1 Diabetes". Diabetes Care (American Diabetes Association) 33 (9): 2010–2012. doi:10.2337/dc10-0404. ISSN 0149-5992. PMID 20551013. 
  6. ↑ Lahner, Edith; Centanni, Marco; Agnello, Giacoma; Gargano, Lucilla; Vannella, Lucy; Iannoni, Carlo; Delle Fave, Gianfranco; Annibale, Bruno (2008). "Occurrence and Risk Factors for Autoimmune Thyroid Disease in Patients with Atrophic Body Gastritis". The American Journal of Medicine (Elsevier BV) 121 (2): 136–141. doi:10.1016/j.amjmed.2007.09.025. ISSN 0002-9343. PMID 18261502. 
  7. ↑ Frommer, Lara; Kahaly, George J (April 26, 2019). "Autoimmune Polyendocrinopathy". The Journal of Clinical Endocrinology & Metabolism (The Endocrine Society) 104 (10): 4769–4782. doi:10.1210/jc.2019-00602. ISSN 0021-972X. PMID 31127843. 

Further reading

  • Betterle, C.; Furmaniak, J.; Sabbadin, C.; Scaroni, C.; Presotto, F. (2023-01-07). "Type 3 autoimmune polyglandular syndrome (APS-3) or type 3 multiple autoimmune syndrome (MAS-3): an expanding galaxy". Journal of Endocrinological Investigation 46 (4): 643–665. doi:10.1007/s40618-022-01994-1. ISSN 1720-8386. PMID 36609775. 
  • Horie, Ichiro; Kawasaki, Eiji; Ando, Takao; Kuwahara, Hironaga; Abiru, Norio; Usa, Toshiro; Yamasaki, Hironori; Ejima, Eri et al. (June 1, 2012). "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): E1043–E1050. doi:10.1210/jc.2011-3109. ISSN 0021-972X. PMID 22466347. 

External links

Classification
External resources



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