Autoimmune polyendocrine syndrome classification: Difference between revisions

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{{Autoimmune polyendocrine syndrome}}
{{Autoimmune polyendocrine syndrome}}
{{CMG}}; {{AE}}
{{CMG}}; {{AE}}{{Akshun}}


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




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{{familytree | | C01 | | | | | C02 | | | | | C03 |C01=Autoimmune polyendocrine syndrome type 1|C02=Autoimmune polyendocrine syndrome type 2|C03=Autoimmune polyendocrine syndrome type 3}}
{{familytree | | C01 | | | | | C02 | | | | | C03 |C01=Autoimmune polyendocrine syndrome type 1|C02=Autoimmune polyendocrine syndrome type 2|C03=Autoimmune polyendocrine syndrome type 3}}
{{familytree | | |!| | | | | | |!| | | | | | |!| }}
{{familytree | | |!| | | | | | |!| | | | | | |!| }}
{{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>}}
{{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>}}
{{familytree | | | | | | | | | | |,|-|-|-|-|-|+|-|-|-|-|-|-|.|}}
{{familytree | | | | | | | | | |F01| | | |F02| | | | | |F03| F01=Autoimmune polyendocrine syndrome type 3A|F02=Autoimmune polyendocrine syndrome type 3B|F03=Autoimmune polyendocrine syndrome type 3C}}
{{familytree | | | | | | | | | | |!| | | | | |!| | | | | | | |!| }}
{{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>}}
{{familytree/end}}
{{familytree/end}}
Autoimmune polyendocrine syndrome (APS) type 3 can be further classified into APS type 3A, APS type 3B and APS type 3C
{{familytree/start}}
{{familytree | | | | | | | | | A01 | | | | | |A01=Autoimmune polyendocrine syndrome type 3}}
{{familytree | | |,|-|-|-|-|-|-|+|-|-|-|-|-|-|.| }}
{{familytree | | C01 | | | | | C02 | | | | | C03 |C01=Autoimmune polyendocrine syndrome type 3A|C02=Autoimmune polyendocrine syndrome type 3B|C03=Autoimmune polyendocrine syndrome type 3C}}
{{familytree | | |!| | | | | | |!| | | | | | |!| }}
{{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>}}
{{familytree/end}}
===Type 2===
''Autoimmune polyendocrine syndrome, type 2'' also known as "Schmidt's syndrome".
Features of this syndrome are:
* Addison's disease
* hypothyroidism
* diabetes mellitus (type 1)
* less common associations:
** hypogonadism
** vitiligo
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.
==Classification==
*There is no established system for the classification of [disease name].
OR
*[Disease name] may be classified according to [classification method] into [number] subtypes/groups:
**[group1]
**[group2]
**[group3]
**[group4]
OR
*[Disease name] may be classified into [large number > 6] subtypes based on:
**[classification method 1]
**[classification method 2]
**[classification method 3]
*[Disease name] may be classified into several subtypes based on:
**[classification method 1]
**[classification method 2]
**[classification method 3]
OR
*Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.
OR
*If the staging system involves specific and characteristic findings and features:
*According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].
OR
*The staging of [malignancy name] is based on the [staging system].
OR
*There is no established system for the staging of [malignancy name].


==References==
==References==

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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