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Key Clinical Updates in Eosinophilic Granulomatosis with Polyangiitis

Mepolizumab, an IL-5 inhibitor, is FDA approved for the treatment of eosinophilic granulomatosis with polyangiitis, although it has not been studied specifically for severe life- or organ-threatening vasculitic disease manifestations (which generally require cyclophosphamide).

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. 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).

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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 Jun;143(6):2170–7.
[PubMed: 30578883]

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