Autoimmune polyendocrine syndrome classification: Difference between revisions

Jump to navigation Jump to search
VisualWikitext
No edit summary
No edit summary
 
(19 intermediate revisions by 3 users not shown)
Line 1: Line 1:
__NOTOC__
__NOTOC__
{{Autoimmune polyendocrine syndrome}}
{{Autoimmune polyendocrine syndrome}}
{{CMG}}; {{AE}}
{{CMG}}; {{AE}}{{Akshun}}


==Overview==
==Overview==
*Autoimmune polyendocrine syndrome can be categorized into three different types namely type 1 {also called autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)}, type 2 and IPEX syndrome.
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]].
 
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], and [group4].
 
OR
 
[Disease name] may be classified into [large number > 6] subtypes based on [classification method 1], [classification method 2], and [classification method 3].
[Disease name] may be classified into several subtypes based on [classification method 1], [classification method 2], and [classification method 3].
 
 
 
 


==Classification==
==Classification==
===Type 1===
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>
{{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].
 
===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].
{{familytree/start}}
OR
{{familytree | | | | | | | | | A01 | | | | | |A01=Autoimmune polyendocrine syndrome (APS)}}
*[Disease name] may be classified according to [classification method] into [number] subtypes/groups:
{{familytree | | |,|-|-|-|-|-|-|+|-|-|-|-|-|-|.| }}
**[group1]
{{familytree | | C01 | | | | | C02 | | | | | C03 |C01=Autoimmune polyendocrine syndrome type 1|C02=Autoimmune polyendocrine syndrome type 2|C03=Autoimmune polyendocrine syndrome type 3}}
**[group2]
{{familytree | | |!| | | | | | |!| | | | | | |!| }}
**[group3]
{{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>}}
**[group4]
{{familytree | | | | | | | | | | |,|-|-|-|-|-|+|-|-|-|-|-|-|.|}}
OR
{{familytree | | | | | | | | | |F01| | | |F02| | | | | |F03| F01=Autoimmune polyendocrine syndrome type 3A|F02=Autoimmune polyendocrine syndrome type 3B|F03=Autoimmune polyendocrine syndrome type 3C}}
*[Disease name] may be classified into [large number > 6] subtypes based on:
{{familytree | | | | | | | | | | |!| | | | | |!| | | | | | | |!| }}
**[classification method 1]
{{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>}}
**[classification method 2]
{{familytree/end}}
**[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

Autoimmune polyendocrine syndrome Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Autoimmune polyendocrine syndrome from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic study of choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Autoimmune polyendocrine syndrome classification On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Autoimmune polyendocrine syndrome classification

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Autoimmune polyendocrine syndrome classification

CDC on Autoimmune polyendocrine syndrome classification

Autoimmune polyendocrine syndrome classification in the news

Blogs on Autoimmune polyendocrine syndrome classification

Directions to Hospitals Treating Autoimmune polyendocrine syndrome

Risk calculators and risk factors for Autoimmune polyendocrine syndrome classification

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.

Template:WH Template:WS

Retrieved from "https://www.wikidoc.org/index.php?title=Autoimmune_polyendocrine_syndrome_classification&oldid=1381012"