{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,29]],"date-time":"2026-01-29T21:48:18Z","timestamp":1769723298597,"version":"3.49.0"},"reference-count":14,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2022,12,17]],"date-time":"2022-12-17T00:00:00Z","timestamp":1671235200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"Oregon Health & Science University Wheels Up Award NIH NCATS","award":["KL2TR002370"],"award-info":[{"award-number":["KL2TR002370"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,6,19]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>This report describes a highly unusual and refractory case of proteinase-3\u2013positive antineutrophil cytoplasmic autoantibody\u2013associated vasculitis, which was associated with marked eosinophilia and only achieved remission after interleukin-5 blockade was added to maximal induction therapy. The patient was a healthy 18-year-old woman who presented with scleritis, otitis, constitutional symptoms, skin and mucosal lesions, and diffuse alveolar haemorrhage and found to have refractory eosinophilia of unclear aetiology. The case did not meet classification criteria for a diagnosis of granulomatosis with polyangiitis or eosinophilic granulomatosis with polyangiitis. Despite induction therapy with glucocorticoids, cyclophosphamide, and rituximab, she did not achieve remission or normalisation of eosinophilia until the addition of mepolizumab, which corresponded to the suppression of peripheral blood eosinophils and clinical remission. This case highlights the limitations of classification criteria and success of interleukin-5 in a multimodal treatment of severe vasculitis with eosinophilia.<\/jats:p>","DOI":"10.1093\/mrcr\/rxac096","type":"journal-article","created":{"date-parts":[[2022,12,17]],"date-time":"2022-12-17T14:01:02Z","timestamp":1671285662000},"page":"431-434","source":"Crossref","is-referenced-by-count":2,"title":["Refractory PR3-positive ANCA-associated vasculitis with eosinophilia requiring anti-IL-5 therapy: A case report"],"prefix":"10.1093","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9925-6644","authenticated-orcid":false,"given":"Elizabeth","family":"Anderson","sequence":"first","affiliation":[{"name":"Department of Medicine, Oregon Health & Science University , Portland, OR, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marcia A","family":"Friedman","sequence":"additional","affiliation":[{"name":"Division of Arthritis and Rheumatic Diseases, School of Medicine, Oregon Health & Science University , Portland, OR, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2022,12,17]]},"reference":[{"key":"2023061920012853100_R1","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1016\/j.jaut.2014.01.028","article-title":"Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener\u2019s granulomatosis)","volume":"48-49","author":"Lutalo","year":"2014","journal-title":"J Autoimmun"},{"key":"2023061920012853100_R2","doi-asserted-by":"crossref","first-page":"622","DOI":"10.1056\/NEJMoa1803537","article-title":"Plasma exchange and glucocorticoids in severe ANCA-associated vasculitis","volume":"382","author":"Walsh","year":"2020","journal-title":"N Engl J Med"},{"key":"2023061920012853100_R3","doi-asserted-by":"crossref","first-page":"1088","DOI":"10.1002\/acr.24634","article-title":"2021 American College of Rheumatology\/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis","volume":"73","author":"Chung","year":"2021","journal-title":"Arthritis Care Res (Hoboken)"},{"key":"2023061920012853100_R4","article-title":"A novel glucocorticoid-free maintenance regimen for anti-neutrophil cytoplasm antibody-associated vasculitis","volume":"58","author":"Pepper","year":"2019","journal-title":"Rheumatology (Oxford)"},{"key":"2023061920012853100_R5","doi-asserted-by":"crossref","first-page":"808","DOI":"10.1080\/02770903.2018.1502301","article-title":"Effects of systemic corticosteroids on blood eosinophil counts in asthma: real-world data","volume":"56","author":"Ortega","year":"2019","journal-title":"J Asthma"},{"key":"2023061920012853100_R6","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1038\/nrrheum.2014.98","article-title":"Eosinophils in vasculitis: characteristics and roles in pathogenesis","volume":"10","author":"Khoury","year":"2014","journal-title":"Nat Rev Rheumatol"},{"key":"2023061920012853100_R7","doi-asserted-by":"crossref","first-page":"1921","DOI":"10.1056\/NEJMoa1702079","article-title":"Mepolizumab or placebo for eosinophilic granulomatosis with polyangiitis","volume":"376","author":"Wechsler","year":"2017","journal-title":"N Engl J Med"},{"key":"2023061920012853100_R8","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1002\/art.41986","article-title":"2022 American College of Rheumatology\/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis","volume":"74","author":"Robson","year":"2022","journal-title":"Arthritis Rheumatol"},{"key":"2023061920012853100_R9","doi-asserted-by":"crossref","first-page":"1094","DOI":"10.1002\/art.1780330806","article-title":"The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis)","volume":"33","author":"Masi","year":"1990","journal-title":"Arthritis Rheum"},{"key":"2023061920012853100_R10","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1136\/annrheumdis-2021-221794","article-title":"2022 American College of Rheumatology\/European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis","volume":"81","author":"Grayson","year":"2022","journal-title":"Ann Rheum Dis"},{"key":"2023061920012853100_R11","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1002\/art.1780370206","article-title":"Nomenclature of systemic vasculitides. 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