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Eosinophilic granulomatosis with polyangiitis (previously called Churg-Strauss syndrome) is an ANCA-associated vasculitis (along with granulomatosis with polyangiitis and microscopic polyangiitis), although the presence of ANCA occurs in less than 50% of patients (usually anti-MPO). It is characterized by peripheral eosinophilia, sinusitis with polyposis, asthma, lung infiltrates, vasculitic skin involvement, glomerulonephritis, and vasculitic neuropathy. Myocarditis can lead to arrhythmias and heart failure if untreated. Eosinophilic granulomatosis with polyangiitis should be considered in patients with an unexplained peripheral eosinophilia and vasculitic features. Laboratory examination of unexplained eosinophilia should include obtaining ANCA and serum tryptase peripheral flow cytometry for PDGF receptor abnormalities and testing for helminthic infections. Eosinophilic infiltrates on tissue samples strongly suggest the diagnosis of eosinophilic granulomatosis, especially if accompanied by vasculitis (purpura, glomerulonephritis, vasculitic ulcers, mononeuritis multiplex). Corticosteroids remain first-line treatment with azathioprine and methotrexate demonstrating efficacy for mild to moderate disease. Mepolizumab, an IL-5 inhibitor, is FDA approved for the treatment of eosinophilic granulomatosis with polyangiitis, although it has not been studied for severe life- or organ-threatening vasculitic disease manifestations (which generally require cyclophosphamide). Rituximab may be considered as an option for patients with refractory disease.

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Canzian  A  et al. Use of biologics to treat relapsing and/or refractory eosinophilic granulomatosis with polyangiitis: data from a European collaborative study. Arthritis Rheumatol. 2021;73:498.
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Steinfeld  J  et al. Evaluation of clinical benefit from treatment with mepolizumab for patients with eosinophilic granulomatosis with polyangiitis. J Allergy Clin Immunol. 2019;143:2170.
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Moiseev  S  et al. International consensus on ANCA testing in eosinophilic granulomatosis with polyangiitis. Am J Respir Crit Care Med. 2020. [Epub ahead of print]
[PubMed: 32584187]

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