Churg-Strauss syndrome pathophysiology

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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. However, further investigations need to be made. [1] [2]

Pathophysiology

Churg–Strauss syndrome[3] 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. Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
  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.

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