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- W3024148437 abstract "Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare but potentially life-threatening antineutrophil cytoplasmic antibody–associated vasculitis that affects, to varying degrees, the lungs, paranasal sinuses, heart, kidneys, skin, and peripheral nervous system. It is strongly associated with asthma. Peripheral eosinophilia is a defining feature of EGPA, and eosinophilic inflammation is often observed in biopsy results of affected tissues. The pathogenesis of EGPA is not completely clear; the disease is probably a result of complex interactions, in which genetic and environmental factors lead to an inflammatory response whose main actors are eosinophils and T and B lymphocytes. 1 Sablé-Fourtassou R. Cohen P. Mahr A. et al. Antineutrophil cytoplasmic antibodies and the Churg–Strauss syndrome. Ann Intern Med. 2005; 143: 632-638 Crossref PubMed Scopus (531) Google Scholar The activated TH2 phenotype triggers the release of key cytokines for the activation, maturation, and survival of eosinophils, such as interleukin (IL)-4, IL-5, and IL-13. 2 Dunogué B. Pagnoux C. Guillevin L. Churg–Strauss syndrome: clinical symptoms, complementary investigations, prognosis and outcome, and treatment. Semin Respir Crit Care Med. 2011; 32: 298-309 Crossref PubMed Scopus (44) Google Scholar In particular, IL-5 is highly increased in active EGPA and its inhibition may represent a potential therapeutic target. 2 Dunogué B. Pagnoux C. Guillevin L. Churg–Strauss syndrome: clinical symptoms, complementary investigations, prognosis and outcome, and treatment. Semin Respir Crit Care Med. 2011; 32: 298-309 Crossref PubMed Scopus (44) Google Scholar ,3 Jakiela B. Szczeklik W. Plutecka H. et al. Increased production of IL-5 and dominant Th2-type response in airways of Churg–Strauss syndrome patients. Rheumatology. 2012; 51: 1887-1893 Crossref PubMed Scopus (77) Google Scholar High eosinophil levels are an integral part of the diagnosis of EGPA and have a cytotoxic and procoagulant effect that may cause the development of cardiovascular and cerebrovascular complications in patients with any type of hypereosinophilic syndrome, including EGPA. Short- and long-term management focuses on the control of inflammation with systemic corticosteroids and other immunosuppressants, all of which carry a substantial burden of adverse effects. The development of monoclonal antibody therapies against IL-5, the major driver of eosinophilic inflammation, has therefore garnered substantial interest among clinicians treating EGPA." @default.
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- W3024148437 date "2020-08-01" @default.
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- W3024148437 title "Eosinophilic granulomatosis with polyangiitis successfully treated with benralizumab" @default.
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- W3024148437 doi "https://doi.org/10.1016/j.anai.2020.05.002" @default.
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