Churg-Strauss syndrome pathophysiology: Difference between revisions

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==Genetics==
==Genetics==
There is correlation between Eosinophilic granulomatosis with polyangiitis with allele HLA-DRB4 and HLA-DRB1*7. However, further investigations need to be made. <ref name="pmid17763415">{{cite journal| author=Vaglio A, Martorana D, Maggiore U, Grasselli C, Zanetti A, Pesci A et al.| title=HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome. | journal=Arthritis Rheum | year= 2007 | volume= 56 | issue= 9 | pages= 3159-66 | pmid=17763415 | doi=10.1002/art.22834 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17763415  }} </ref> <ref name="pmid18163478">{{cite journal| author=Wieczorek S, Hellmich B, Gross WL, Epplen JT| title=Associations of Churg-Strauss syndrome with the HLA-DRB1 locus, and relationship to the genetics of antineutrophil cytoplasmic antibody-associated vasculitides: comment on the article by Vaglio et al. | journal=Arthritis Rheum | year= 2008 | volume= 58 | issue= 1 | pages= 329-30 | pmid=18163478 | doi=10.1002/art.23209 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18163478  }} </ref>
There is correlation between Eosinophilic granulomatosis with polyangiitis with allele HLA-DRB4 and HLA-DRB1*7.<ref name="pmid17763415">{{cite journal| author=Vaglio A, Martorana D, Maggiore U, Grasselli C, Zanetti A, Pesci A et al.| title=HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome. | journal=Arthritis Rheum | year= 2007 | volume= 56 | issue= 9 | pages= 3159-66 | pmid=17763415 | doi=10.1002/art.22834 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17763415  }} </ref> <ref name="pmid18163478">{{cite journal| author=Wieczorek S, Hellmich B, Gross WL, Epplen JT| title=Associations of Churg-Strauss syndrome with the HLA-DRB1 locus, and relationship to the genetics of antineutrophil cytoplasmic antibody-associated vasculitides: comment on the article by Vaglio et al. | journal=Arthritis Rheum | year= 2008 | volume= 58 | issue= 1 | pages= 329-30 | pmid=18163478 | doi=10.1002/art.23209 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18163478  }} </ref> Presence of elevated HLA-DRB4 gene in patients with asthma (with or without eosinophilia), could be used to identify a possible risk of developing Eosinophilc granulomatosis with polyangiitis. However, further follow up studies are required. <ref name="pmid24734195">{{cite journal| author=Bottero P, Motta F, Bonini M, Vecchio F, Ierna F, Cuppari I et al.| title=Can HLA-DRB4 Help to Identify Asthmatic Patients at Risk of Churg-Strauss Syndrome? | journal=ISRN Rheumatol | year= 2014 | volume= 2014 | issue=  | pages= 843804 | pmid=24734195 | doi=10.1155/2014/843804 | pmc=3963189 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24734195  }}</ref>
==Pathophysiology==
==Pathophysiology==
'''Churg–Strauss syndrome'''<ref name="Bolognia">{{cite book |author=Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. |title=Dermatology: 2-Volume Set |publisher=Mosby |location=St. Louis |year=2007 |pages= |isbn=1-4160-2999-0 |oclc= |doi= |accessdate=}}</ref> is a medium and small vessel [[autoimmune disease|autoimmune]] [[vasculitis]], leading to [[necrosis]].  It involves mainly the blood vessels of the [[lungs]] (it begins as a severe type of [[asthma]]), gastrointestinal system, and peripheral nerves, but also affects the heart, skin, and kidneys.
'''Churg–Strauss syndrome'''<ref name="Bolognia">{{cite book |author=Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. |title=Dermatology: 2-Volume Set |publisher=Mosby |location=St. Louis |year=2007 |pages= |isbn=1-4160-2999-0 |oclc= |doi= |accessdate=}}</ref> is a medium and small vessel [[autoimmune disease|autoimmune]] [[vasculitis]], leading to [[necrosis]].  It involves mainly the blood vessels of the [[lungs]] (it begins as a severe type of [[asthma]]), gastrointestinal system, and peripheral nerves, but also affects the heart, skin, and kidneys.

Revision as of 15:35, 18 November 2016

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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

Pathogenesis

The pathogenesis of Eosinophilic granulomatosis with polyangiitis is not fully understood. However, it is hypothesized

Genetics

There is correlation between Eosinophilic granulomatosis with polyangiitis with allele HLA-DRB4 and HLA-DRB1*7.[1] [2] Presence of elevated HLA-DRB4 gene in patients with asthma (with or without eosinophilia), could be used to identify a possible risk of developing Eosinophilc granulomatosis with polyangiitis. However, further follow up studies are required. [3]

Pathophysiology

Churg–Strauss syndrome[4] is a medium and small vessel autoimmune vasculitis, leading to necrosis. It involves mainly the blood vessels of the lungs (it begins as a severe type of asthma), gastrointestinal system, and peripheral nerves, but also affects the heart, skin, and kidneys.

Associated Conditions

The following conditions are associated with Eosinophilic granulomatosis with polyangiitis:

Microscopic Pathology

  • Eosinophilic infiltrates with necrosis (sometimes quite extensive)
  • Giant cell vasculitis with eosinophils
  • Interstitial and perivascular necrotizing granulomas
  • Eosinophilic lymphadenopathy
Necrotizing and granulomatous arteritis in the lung of a patient with Churg-Strauss syndrome.

References

  1. Vaglio A, Martorana D, Maggiore U, Grasselli C, Zanetti A, Pesci A; et al. (2007). "HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome". Arthritis Rheum. 56 (9): 3159–66. doi:10.1002/art.22834. PMID 17763415.
  2. Wieczorek S, Hellmich B, Gross WL, Epplen JT (2008). "Associations of Churg-Strauss syndrome with the HLA-DRB1 locus, and relationship to the genetics of antineutrophil cytoplasmic antibody-associated vasculitides: comment on the article by Vaglio et al". Arthritis Rheum. 58 (1): 329–30. doi:10.1002/art.23209. PMID 18163478.
  3. Bottero P, Motta F, Bonini M, Vecchio F, Ierna F, Cuppari I; et al. (2014). "Can HLA-DRB4 Help to Identify Asthmatic Patients at Risk of Churg-Strauss Syndrome?". ISRN Rheumatol. 2014 ( ): 843804. doi:10.1155/2014/843804. PMC 3963189. PMID 24734195.
  4. Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
  5. Lee SH, Roh MR, Jee H, Chung KY, Jung JY (2011). "Wells' syndrome associated with churg-strauss syndrome". Ann Dermatol. 23 (4): 497–500. doi:10.5021/ad.2011.23.4.497. PMC 3229945. PMID 22148019.
  6. Maamar M, Tazi-Mezalek Z, Harmouche H, El Hamany Z, Adnaoui M, Aouni M (2012). "Churg-Strauss syndrome associated with AA amyloidosis: a case report". Pan Afr Med J. 12: 30. PMC 3415051. PMID 22891088.

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