{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T07:47:58Z","timestamp":1791445678807,"version":"4.3.3"},"reference-count":39,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2021,1,29]],"date-time":"2021-01-29T00:00:00Z","timestamp":1611878400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["ACR Open Rheumatology"],"published-print":{"date-parts":[[2021,2]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>Eosinophilic granulomatosis with polyangiitis (EGPA) is part of a group of vasculitides commonly referred to as antineutrophil cytoplasmic antibody (ANCA)\u2013associated vasculitis (AAV), in addition to granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and renal\u2010limited vasculitis. Patients with EGPA characteristically have asthma and marked peripheral eosinophilia with only approximately 30% to 35% of patients being myeloperoxidase (MPO)\u2010ANCA positive, distinguishing it from other forms of AAV (1,2). The aim of this systematic review is to support the development of the American College of Rheumatology\/Vasculitis Foundation guideline for the management of EGPA.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A systematic review was conducted of the literature for seven forms of primary systemic vasculitis (GPA, MPA, EGPA, polyarteritis nodosa, Kawasaki disease, giant cell arteritis, and Takayasu arteritis). The search was done for articles in English using Ovid Medline, PubMed, Embase, and the Cochrane Library. Articles were screened for suitability in addressing population\/patients, intervention, comparator, and outcomes (PICO) questions, with studies presenting the highest level of evidence given preference. Two independent reviewers conducted a title\/abstract screen and full\u2010text review for each eligible study.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The initial search, conducted in August 2019, included 13\u2009800 articles, of which 2596 full\u2010text articles were reviewed. There were 190 articles (addressing 34 PICO questions) reporting on the diagnosis and management of EGPA.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This comprehensive systematic review synthesizes and evaluates the accuracy of commonly used tests for EGPA as well as benefits and toxicities of different treatment options.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr2.11194","type":"journal-article","created":{"date-parts":[[2021,1,29]],"date-time":"2021-01-29T11:12:20Z","timestamp":1611918740000},"page":"101-110","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["Eosinophilic Granulomatosis with Polyangiitis: A Systematic Review and Meta\u2010Analysis of Test Accuracy and Benefits and Harms of Common Treatments"],"prefix":"10.1002","volume":"3","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3903-6049","authenticated-orcid":false,"given":"Jason M.","family":"Springer","sequence":"first","affiliation":[{"name":"Vanderbilt University Medical Center Nashville"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6581-4561","authenticated-orcid":false,"given":"Mohamad A.","family":"Kalot","sequence":"additional","affiliation":[{"name":"State University of New York at Buffalo"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9638-2992","authenticated-orcid":false,"given":"Nedaa M.","family":"Husainat","sequence":"additional","affiliation":[{"name":"St. Mary's Hospital St. Louis Missouri"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6441-1915","authenticated-orcid":false,"given":"Kevin W.","family":"Byram","sequence":"additional","affiliation":[{"name":"Vanderbilt University Medical Center Nashville"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3508-9290","authenticated-orcid":false,"given":"Anisha B.","family":"Dua","sequence":"additional","affiliation":[{"name":"Northwestern University Feinberg School of Medicine Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen E.","family":"James","sequence":"additional","affiliation":[{"name":"University of Utah Health Salt Lake City"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yih","family":"Chang Lin","sequence":"additional","affiliation":[{"name":"University of South Florida Tampa"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marat","family":"Turgunbaev","sequence":"additional","affiliation":[{"name":"American College of Rheumatology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Villa\u2010Forte","sequence":"additional","affiliation":[{"name":"Cleveland Clinic Cleveland Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andy","family":"Abril","sequence":"additional","affiliation":[{"name":"Mayo Clinic Jacksonville Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carol A.","family":"Langford","sequence":"additional","affiliation":[{"name":"Cleveland Clinic Cleveland Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mehrdad","family":"Maz","sequence":"additional","affiliation":[{"name":"University of Kansas Medical Center Kansas City"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sharon A.","family":"Chung","sequence":"additional","affiliation":[{"name":"University of California, San Francisco Medical Center"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2091-0875","authenticated-orcid":false,"given":"Reem A.","family":"Mustafa","sequence":"additional","affiliation":[{"name":"University of Kansas Medical Center Kansas City"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,1,29]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.21250"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-143-9-200511010-00006"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202005-1628SO"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-198403000-00001"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780330806"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.37715"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rdc.2010.04.002"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-199601000-00003"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.md.0000180793.80212.17"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2012-201531"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2013.03.003"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/1756-185X.13056"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0b013e318205a4c6"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.23912"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejim.2016.09.014"},{"key":"e_1_2_7_17_1","first-page":"51","article-title":"The significance of cardiac magnetic resonance imaging in detection and monitoring of the treatment efficacy of heart involvement in eosinophilic granulomatosis with polyangiitis patients","volume":"33","author":"Fijolek J","year":"2016","journal-title":"Sarcoidosis Vasc Diffuse Lung Dis"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10554-016-0843-y"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2015.06.087"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.2174\/18715281113129990054"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1253\/circj.CJ-10-0772"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.27263"},{"issue":"52","key":"e_1_2_7_23_1","first-page":"S70","article-title":"Impact of cardiac magnetic resonance imaging for assessment of Churg\u2010Strauss syndrome: a cross\u2010sectional study in 20 patients","volume":"27","author":"Marmursztejn J","year":"2009","journal-title":"Clin Exp Rheumatol"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0b013e3181af35a5"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cardfail.2008.07.227"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2015.04.013"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.23198"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.39011"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.22679"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaip.2017.07.027"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2014-206095"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1136\/rmdopen-2019-000905"},{"key":"e_1_2_7_33_1","doi-asserted-by":"crossref","first-page":"952","DOI":"10.1136\/annrheumdis-2017-211897","article-title":"Scheduled rituximab maintenance reduces relapse rate in eosinophilic granulomatosis with polyangiitis","volume":"77","author":"Emmi G","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"e_1_2_7_34_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1702079"},{"issue":"36","key":"e_1_2_7_35_1","first-page":"S52","article-title":"Churg Strauss syndrome\u2013successful induction of remission with methotrexate and unexpected high cardiac and pulmonary relapse ratio during maintenance treatment","volume":"22","author":"Metzler C","year":"2004","journal-title":"Clin Exp Rheumatol"},{"key":"e_1_2_7_36_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0185880"},{"key":"e_1_2_7_37_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.40205"},{"key":"e_1_2_7_38_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2017-211796"},{"key":"e_1_2_7_39_1","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2007.087825"},{"key":"e_1_2_7_40_1","doi-asserted-by":"publisher","DOI":"10.1002\/pds.1353"}],"container-title":["ACR Open Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/acr2.11194","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/acr2.11194","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/acr2.11194","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,2]],"date-time":"2025-10-02T16:13:05Z","timestamp":1759421585000},"score":1,"resource":{"primary":{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/10.1002\/acr2.11194"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,1,29]]},"references-count":39,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2021,2]]}},"alternative-id":["10.1002\/acr2.11194"],"URL":"https:\/\/doi.org\/10.1002\/acr2.11194","archive":["Portico"],"relation":{},"ISSN":["2578-5745","2578-5745"],"issn-type":[{"value":"2578-5745","type":"print"},{"value":"2578-5745","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,1,29]]},"assertion":[{"value":"2020-05-28","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-10-07","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-01-29","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}