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{{Autoimmune polyendocrine syndrome}}
{{Autoimmune polyendocrine syndrome}}
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==Overview==
==Overview==
Autoimmune polyendocrine syndrome (APS) can be classified into APS type 1, APS type 2 and APS type 3 on the basis of organ involvement. APS type 1 commonly presents with mucocutaneous [[candidiasis]], [[hypoparathyroidism]] and [[Addison's disease]]. APS type 2  most commonly presents with [[Addison's disease]], [[autoimmune thyroiditis]] and [[diabetes mellitus type 1]]. APS type 3 usually presents with [[autoimmune thyroiditis]], [[diabetes mellitus type 1]] and [[pernicious anemia]].


==Classification==
==Classification==
On the basis of [[Organ (anatomy)|organ]] involvement, autoimmune polyendocrine syndrome (APS) can be classified into APS type 1, APS type 2 and APS type 3.<ref name="pmid12817789">{{cite journal |vauthors=Betterle C, Zanchetta R |title=Update on autoimmune polyendocrine syndromes (APS) |journal=Acta Biomed |volume=74 |issue=1 |pages=9–33 |year=2003 |pmid=12817789 |doi= |url=}}</ref><ref name="pmid5067225">{{cite journal |vauthors=Forcier RJ, McIntyre OR, Frey WG, Andrada JA, Streiff RR |title=Autoimmunity and multiple endocrine abnormalities |journal=Arch. Intern. Med. |volume=129 |issue=4 |pages=638–41 |year=1972 |pmid=5067225 |doi= |url=}}</ref><ref name="pmid15141045">{{cite journal |vauthors=Eisenbarth GS, Gottlieb PA |title=Autoimmune polyendocrine syndromes |journal=N. Engl. J. Med. |volume=350 |issue=20 |pages=2068–79 |year=2004 |pmid=15141045 |doi=10.1056/NEJMra030158 |url=}}</ref>


=== Type 1 ===
{{Seealso|Autoimmune polyendocrine syndrome type 1}}
''Autoimmune polyendocrine syndrome, type 1'' is also known as the [[candidiasis]]-[[hypoparathyroidism]]-[[Addison's disease]]-syndrome after its main features:
* A mild [[immune deficiency]], leading to persistent mucosal and cutaneous infections with [[Candida (genus)|candida]] yeasts. There is also decreased function of the [[spleen]] (asplenism).
* Autoimmune dysfunction of the [[parathyroid gland]] (leading to [[hypocalcemia]]) and the [[adrenal gland]] (Addison's disease: [[hypoglycemia]], [[hypotension]] and severe reactions in disease).
* Other disease associations are:
** [[hypothyroidism]]
** [[hypogonadism]] and infertility
** [[diabetes mellitus]] (type 1)
** [[vitiligo]] (depigmentation of the skin)
** [[alopecia]] (baldness)
** [[malabsorption]]
** [[pernicious anemia]]
** [[hepatitis#autoimmune|chronic active (autoimmune) hepatitis]]


A EU-funded consortium is currently doing translational research on this condition and has establised w webpage at [http://www.apeced.net EurAPS].
{{familytree/start}}
 
{{familytree | | | | | | | | | A01 | | | | | |A01=Autoimmune polyendocrine syndrome (APS)}}
=== Type 2 ===
{{familytree | | |,|-|-|-|-|-|-|+|-|-|-|-|-|-|.| }}
''Autoimmune polyendocrine syndrome, type 2'' also known as "Schmidt's syndrome".
{{familytree | | C01 | | | | | C02 | | | | | C03 |C01=Autoimmune polyendocrine syndrome type 1|C02=Autoimmune polyendocrine syndrome type 2|C03=Autoimmune polyendocrine syndrome type 3}}
Features of this syndrome are:
{{familytree | | |!| | | | | | |!| | | | | | |!| }}
* Addison's disease
{{familytree | | D01 | | | | | D02 | | | | | D03 |D01=•Mucocutaneous [[candidiasis]]<br> •[[Hypoparathyroidism]]<br> •[[Addison's disease]]<br>|D02=•[[Addison's disease]]<br>•[[Autoimmune thyroiditis]]<br> •[[Diabetes mellitus type 1]]|D03=•[[Autoimmune thyroiditis]]<br> •[[Diabetes mellitus type 1]]<br> •[[Pernicious anemia]]<br>}}
* hypothyroidism
{{familytree | | | | | | | | | | |,|-|-|-|-|-|+|-|-|-|-|-|-|.|}}
* diabetes mellitus (type 1)
{{familytree | | | | | | | | | |F01| | | |F02| | | | | |F03| F01=Autoimmune polyendocrine syndrome type 3A|F02=Autoimmune polyendocrine syndrome type 3B|F03=Autoimmune polyendocrine syndrome type 3C}}
* less common associations:
{{familytree | | | | | | | | | | |!| | | | | |!| | | | | | | |!| }}
** hypogonadism
{{familytree | | | | | | | | | |D01 | | | |D02 | | | | | | D03 |D01=•[[Autoimmune thyroiditis]]<br> •Immune mediated [[diabetes mellitus]]<br>|D02=•[[Autoimmune thyroiditis]]<br>•[[Pernicious anemia]]<br>|D03=•[[Autoimmune thyroiditis]]<br>•[[Vitiligo]]/[[Alopecia]]<br>}}
** vitiligo
{{familytree/end}}
 
Some researchers favour splitting this syndrome into three distinct syndromes (numbering 2, 3 and 4), but research evidence for these distinct combinations is not convincing.
 
=== XPID ===
The most serious but rarest form is the ''X-linked polyendocrinopathy, immunodeficiency and [[diarrhea]]''-syndrome, also called [[IPEX]].  Most patients develop diabetes and diarrhea as neonates and many die due to autoimmune activity against many organs. Boys are affected, while girls are carriers and might suffer mild disease.
 
=== Other diseases ===
Other diseases featuring polyendocrine autoimmunity:
* Chromosomal abnormalities ([[Down's syndrome]]) increase the risk of endocrine autoimmunity
* POEMS syndrome - the ''E'' is for endocrinopathy; the cause is a [[paraprotein]] excreted by a plasmacytoma or [[multiple myeloma]]; other features are [[polyneuropathy]], organomegaly ([[hepatomegaly]] and [[splenomegaly]]), M-protein (paraprotein) and skin changes.
* Several very [[rare disease]]s.


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
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{{WS}}
[[Category:Endocrine system]]
[[Category:Endocrinology]]
[[Category:Overview complete]]
[[Category:Syndromes]]
[[Category:Autoimmune diseases]]
 
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Latest revision as of 19:51, 27 October 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Akshun Kalia M.B.B.S.[2]

Overview

Autoimmune polyendocrine syndrome (APS) can be classified into APS type 1, APS type 2 and APS type 3 on the basis of organ involvement. APS type 1 commonly presents with mucocutaneous candidiasis, hypoparathyroidism and Addison's disease. APS type 2 most commonly presents with Addison's disease, autoimmune thyroiditis and diabetes mellitus type 1. APS type 3 usually presents with autoimmune thyroiditis, diabetes mellitus type 1 and pernicious anemia.

Classification

On the basis of organ involvement, autoimmune polyendocrine syndrome (APS) can be classified into APS type 1, APS type 2 and APS type 3.[1][2][3]


 
 
 
 
 
 
 
 
Autoimmune polyendocrine syndrome (APS)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Autoimmune polyendocrine syndrome type 1
 
 
 
 
Autoimmune polyendocrine syndrome type 2
 
 
 
 
Autoimmune polyendocrine syndrome type 3
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
•Mucocutaneous candidiasis
•Hypoparathyroidism
•Addison's disease
 
 
 
 
•Addison's disease
•Autoimmune thyroiditis
•Diabetes mellitus type 1
 
 
 
 
•Autoimmune thyroiditis
•Diabetes mellitus type 1
•Pernicious anemia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Autoimmune polyendocrine syndrome type 3A
 
 
 
Autoimmune polyendocrine syndrome type 3B
 
 
 
 
 
Autoimmune polyendocrine syndrome type 3C
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
•Autoimmune thyroiditis
•Immune mediated diabetes mellitus
 
 
 
•Autoimmune thyroiditis
•Pernicious anemia
 
 
 
 
 
•Autoimmune thyroiditis
•Vitiligo/Alopecia

References

  1. ↑ Betterle C, Zanchetta R (2003). "Update on autoimmune polyendocrine syndromes (APS)". Acta Biomed. 74 (1): 9–33. PMID 12817789.
  2. ↑ Forcier RJ, McIntyre OR, Frey WG, Andrada JA, Streiff RR (1972). "Autoimmunity and multiple endocrine abnormalities". Arch. Intern. Med. 129 (4): 638–41. PMID 5067225.
  3. ↑ Eisenbarth GS, Gottlieb PA (2004). "Autoimmune polyendocrine syndromes". N. Engl. J. Med. 350 (20): 2068–79. doi:10.1056/NEJMra030158. PMID 15141045.

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