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{{Churg-Strauss syndrome}}
{{Churg-Strauss syndrome}}
{{CMG}}; {{AE}} {{CK}}  
{{CMG}}{{APM}}{{AE}}{{KW}}
==Overview==
==Overview==
The exact pathogenesis of [disease name] is not fully understood.
According to the American College of Rheumatology (ACR), Eosinophilic granulomatosis with polyangiitis is classified using 6 criteria.  


OR
==Classification==
According to the 2012 Revised International Chapel Hill Consensus Conference on Nomenclature of Vasculitides<ref name="pmid23045170">{{cite journal| author=Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F et al.| title=2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. | journal=Arthritis Rheum | year= 2013 | volume= 65 | issue= 1 | pages= 1-11 | pmid=23045170 | doi=10.1002/art.37715 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23045170  }} </ref>, Eosinophilic Granulomatosis with Polyangitis (formerly known as Churg-Strauss' syndrome) can be considered a variant of the ANCA associated vasculitides of the small vessels. The Chapel Hill Consensus Conference however does not propose any diagnostic or classification criteria. Therefore, the diagnostic criteria proposed by the American College of Rheumatology (ACR) in 1990 on Eosinophilic granulomatosis with polyangiitis is still being used to distinguish the disease.   


It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
The presence of 4 out of the 6 diagnostic criteria has a reported sensitivity of 85% and a specificity of 99.7% for the diagnosis of  Eosinophilic granulomatosis with polyangiitis<ref name="pmid2202307">{{cite journal| author=Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP et al.| title=The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). | journal=Arthritis Rheum | year= 1990 | volume= 33 | issue= 8 | pages= 1094-100 | pmid=2202307 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2202307  }}</ref><ref name="pmid25404930">{{cite journal| author=Gioffredi A, Maritati F, Oliva E, Buzio C| title=Eosinophilic granulomatosis with polyangiitis: an overview. | journal=Front Immunol | year= 2014 | volume= 5 | issue= | pages= 549 | pmid=25404930 | doi=10.3389/fimmu.2014.00549 | pmc=4217511 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25404930  }} </ref>. They are: 
 
{| class="wikitable"
OR
!American College of Rheumatology (ACR) Classification of Eosinophilic granulomatosis with polyangiitis
 
|-
[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
|Asthma
 
|-
OR
|Eosinophilia > 10%
 
|-
Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
|Neuropathy (mononeuropathy or polyneuropathy)
 
|-
OR
|Pulmonary infiltrate, non fixed
 
|-
 
|Paranasal sinus abnormality
[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
|-
 
|Biopsy that shows extravascular eosinophil infiltration
OR
|}
 
4 out of the 6 criteria are needed to distinguish Eosinophilic granulomatosis with polyangiitis from other vasculitides.  
The progression to [disease name] usually involves the [molecular pathway].
 
OR
 
The pathophysiology of [disease/malignancy] depends on the histological subtype.
 
==Pathophysiology==
 
===Pathogenesis===
*The exact pathogenesis of [disease name] is not fully understood.
OR
*It is understood that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
*[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
*Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
*[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
*The progression to [disease name] usually involves the [molecular pathway].
*The pathophysiology of [disease/malignancy] depends on the histological subtype.
 
==Genetics==
*HLA-DRB1*7, and HLA-DRB4 are associated with the development of eosinophilic granulomatosis with polyangiitis. HLA-DRB4 is correlated with increasing risk of development of vascular manifestations of the churg-strauss syndrome.<ref name="pmid17763415">{{cite journal |vauthors=Vaglio A, Martorana D, Maggiore U, Grasselli C, Zanetti A, Pesci A, Garini G, Manganelli P, Bottero P, Tumiati B, Sinico RA, Savi M, Buzio C, Neri TM |title=HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome |journal=Arthritis Rheum. |volume=56 |issue=9 |pages=3159–66 |date=September 2007 |pmid=17763415 |doi=10.1002/art.22834 |url=}}</ref><ref name="pmid24734195">{{cite journal |vauthors=Bottero P, Motta F, Bonini M, Vecchio F, Ierna F, Cuppari I, Sinico RA |title=Can HLA-DRB4 Help to Identify Asthmatic Patients at Risk of Churg-Strauss Syndrome? |journal=ISRN Rheumatol |volume=2014 |issue= |pages=843804 |date=2014 |pmid=24734195 |pmc=3963189 |doi=10.1155/2014/843804 |url=}}</ref>
*Single-nucleotide polymorphisms in the Interleukin-10 gene (IL10.2 haplotype) have been associated with eosinophilic granulomatosis with polyangitis.<ref name="pmid18512809">{{cite journal |vauthors=Wieczorek S, Hellmich B, Arning L, Moosig F, Lamprecht P, Gross WL, Epplen JT |title=Functionally relevant variations of the interleukin-10 gene associated with antineutrophil cytoplasmic antibody-negative Churg-Strauss syndrome, but not with Wegener's granulomatosis |journal=Arthritis Rheum. |volume=58 |issue=6 |pages=1839–48 |date=June 2008 |pmid=18512809 |doi=10.1002/art.23496 |url=}}</ref>
 
==Associated Conditions==
 
==Gross Pathology==
*On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
 
==Microscopic Pathology==
*On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
[[Category:Disease]]
[[Category:Pulmonology]]
[[Category:Autoimmune diseases]]
[[Category:Rheumatology]]
[[Category:Needs content]]


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{{WS}}
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Revision as of 18:38, 29 March 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [3]

Overview

According to the American College of Rheumatology (ACR), Eosinophilic granulomatosis with polyangiitis is classified using 6 criteria.

Classification

According to the 2012 Revised International Chapel Hill Consensus Conference on Nomenclature of Vasculitides[1], Eosinophilic Granulomatosis with Polyangitis (formerly known as Churg-Strauss' syndrome) can be considered a variant of the ANCA associated vasculitides of the small vessels. The Chapel Hill Consensus Conference however does not propose any diagnostic or classification criteria. Therefore, the diagnostic criteria proposed by the American College of Rheumatology (ACR) in 1990 on Eosinophilic granulomatosis with polyangiitis is still being used to distinguish the disease.

The presence of 4 out of the 6 diagnostic criteria has a reported sensitivity of 85% and a specificity of 99.7% for the diagnosis of Eosinophilic granulomatosis with polyangiitis[2][3]. They are:

American College of Rheumatology (ACR) Classification of Eosinophilic granulomatosis with polyangiitis
Asthma
Eosinophilia > 10%
Neuropathy (mononeuropathy or polyneuropathy)
Pulmonary infiltrate, non fixed
Paranasal sinus abnormality
Biopsy that shows extravascular eosinophil infiltration

4 out of the 6 criteria are needed to distinguish Eosinophilic granulomatosis with polyangiitis from other vasculitides.

References

  1. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F; et al. (2013). "2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides". Arthritis Rheum. 65 (1): 1–11. doi:10.1002/art.37715. PMID 23045170.
  2. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP; et al. (1990). "The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis)". Arthritis Rheum. 33 (8): 1094–100. PMID 2202307.
  3. Gioffredi A, Maritati F, Oliva E, Buzio C (2014). "Eosinophilic granulomatosis with polyangiitis: an overview". Front Immunol. 5: 549. doi:10.3389/fimmu.2014.00549. PMC 4217511. PMID 25404930.

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