Churg-Strauss syndrome pathophysiology: Difference between revisions

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==Genetics==
==Genetics==
The allele HLA-DRB1*7 and HLA-DRB4 is correlated with Eosinophilic granulomatosis with polyangiitis.<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>
*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==
==Associated Conditions==

Revision as of 20:14, 27 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

Pathogenesis

The pathogenesis of Eosinophilic granulomatosis with polyangiitis is not fully understood. Eosinophilic granulomatosis with polyangiitis [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.

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.[1][2]
  • Single-nucleotide polymorphisms in the Interleukin-10 gene (IL10.2 haplotype) have been associated with eosinophilic granulomatosis with polyangitis.[3]

Associated Conditions

The following conditions are associated with Eosinophilic granulomatosis with polyangiitis:

Gross Pathology

On gross pathology, the following changes are typically seen:[6]

  • Nodular swelling that appears along the small arteries of various organs, such as the heart, liver, and the renal
  • Infarcts, hemorrhage and scarring of affected organs
  • Pulmonary artery occlusion
  • Patchy consolidations commonly affixed in the lower portions of the lung
  • Fibrosis, ventricular hypertrophy of both ventricles, patchy myocardial scars, and endocardial fibrosis of the heart

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, Garini G, Manganelli P, Bottero P, Tumiati B, Sinico RA, Savi M, Buzio C, Neri TM (September 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. Bottero P, Motta F, Bonini M, Vecchio F, Ierna F, Cuppari I, Sinico RA (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.
  3. Wieczorek S, Hellmich B, Arning L, Moosig F, Lamprecht P, Gross WL, Epplen JT (June 2008). "Functionally relevant variations of the interleukin-10 gene associated with antineutrophil cytoplasmic antibody-negative Churg-Strauss syndrome, but not with Wegener's granulomatosis". Arthritis Rheum. 58 (6): 1839–48. doi:10.1002/art.23496. PMID 18512809.
  4. 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.
  5. 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.
  6. CHURG J, STRAUSS L (1951). "Allergic granulomatosis, allergic angiitis, and periarteritis nodosa". Am J Pathol. 27 (2): 277–301. PMC 1937314. PMID 14819261.

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