Churg-Strauss syndrome pathophysiology: Difference between revisions

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==Pathogenesis==
==Pathogenesis==
The pathogenesis of Eosinophilic granulomatosis with polyangiitis is not fully understood.
The pathogenesis of Eosinophilic granulomatosis with polyangiitis is not fully understood. It is hypothesized that the following outcomes attribute to the development of the disease:


==Genetics==
==Genetics==

Revision as of 13:24, 21 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. It is hypothesized that the following outcomes attribute to the development of the disease:

Genetics

The allele HLA-DRB1*7 and HLA-DRB4 is correlated with Eosinophilic granulomatosis with polyangiitis.[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:

Gross Pathology

On gross pathology, the following changes are typically seen:[7]

  • 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; 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.
  7. 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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