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Sinusitis 2016, 1(1), 24-43; doi:10.3390/sinusitis1010024

Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis

1
Department of Pneumology and Allergy, Immunoallergia Respiratoria Clinica I Experimental, Hospital Clinic, 08950 Barcelona, Spain
2
Clínica Diagonal, 08950 Barcelona, Spain
3
Rhinology Unit and Smell Clinic, Department of Otorhinolaryngology, Hospital Clinic, University of Barcelona, 08950 Barcelona, Spain
4
Laboratory of Clinical and Experimental Respiratory Immunoallergy, IDIBAPS, 08950 Barcelona, Spain
*
Author to whom correspondence should be addressed.
Academic Editor: César Picado
Received: 31 October 2015 / Revised: 2 December 2015 / Accepted: 14 December 2015 / Published: 17 December 2015
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Abstract

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss, EGPA) is a systemic small-to-medium-sized vasculitis associated with asthma and eosinophilia. Histologically EGPA presents tissue eosinophilia, necrotizing vasculitis, and granulomatous inflammation with eosinophil tissue infiltration. EGPA commonly involves the upper airway and lung parenchyma, peripheral neuropathy, cardiac disorders, and skin lesions. The anti-neutrophil cytoplasmic antibodies (ANCA) are positive in 40% of cases, especially in those patients with clinical signs of vasculitis. The pathogenesis of EGPA is multifactorial. The disease can be triggered by exposure to a variety of allergens and drugs, but a genetic background has also been described, particularly an association with HLA-DRB4. Th2 response is of special importance in the upregulation of different interleukins such as IL-4, IL-13, and IL-5. Th1 and Th17 responses are also of significance. Activated eosinophils have a prolonged survival and probably cause tissue damage by releasing eosinophil granule proteins, while their tissue recruitment can be regulated by chemokines such as eotaxin-3 and CCL17. Humoral immunity is also abnormally regulated, as demonstrated by excessive responses of IgG4 and IgE. EGPA has a good respond to glucocorticoids, although the combination of glucocorticoids and immunosuppressants (e.g., cyclophosphamide, azathioprine) is needed in most of cases. Newer treatment options include anti-IL-5 antibodies (mepolizumab), whose efficacy has been described in clinical trials, and anti-CD-20, a B cell-depleting agent (rituximab), reported in several case series. View Full-Text
Keywords: eosinophilic granulomatosis with polyangiitis; vasculitis; asthma; eosinophilia; glucocorticoids eosinophilic granulomatosis with polyangiitis; vasculitis; asthma; eosinophilia; glucocorticoids
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MDPI and ACS Style

Izquierdo-Domínguez, A.; Cordero Castillo, A.; Alobid, I.; Mullol, J. Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis. Sinusitis 2016, 1, 24-43.

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