{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T11:57:22Z","timestamp":1791633442944,"version":"4.3.4"},"reference-count":11,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2022,2,2]],"date-time":"2022-02-02T00:00:00Z","timestamp":1643760000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000960","name":"ACR","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000960","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008741","name":"European League Against Rheumatism","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100008741","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007211","name":"Vasculitis Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007211","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2022,3]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To develop and validate revised classification criteria for eosinophilic granulomatosis with polyangiitis (EGPA).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in 5 phases: 1) identification of candidate criteria items using consensus methodology, 2) prospective collection of candidate items present at the time of diagnosis, 3) data\u2010driven reduction of the number of candidate items, 4) expert panel review of cases to define the reference diagnosis, and 5) derivation of a points\u2010based risk score for disease classification in a development set using least absolute shrinkage and selection operator logistic regression, with subsequent validation of performance characteristics in an independent set of cases and comparators.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The development set for EGPA consisted of 107 cases of EGPA and 450 comparators. The validation set consisted of an additional 119 cases of EGPA and 437 comparators. From 91 candidate items, regression analysis identified 11 items for EPGA, 7 of which were retained. The final criteria and their weights were as follows: maximum eosinophil count \u22651\u2009\u00d7\u200910\n                      <jats:sup>9<\/jats:sup>\n                      \/liter (+5), obstructive airway disease (+3), nasal polyps (+3), cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or anti\u2013proteinase 3 ANCA positivity (\u22123), extravascular eosinophilic predominant inflammation (+2), mononeuritis multiplex\/motor neuropathy not due to radiculopathy (+1), and hematuria (\u22121). After excluding mimics of vasculitis, a patient with a diagnosis of small\u2010 or medium\u2010vessel vasculitis could be classified as having EGPA if the cumulative score was \u22656 points. When these criteria were tested in the validation data set, the sensitivity was 85% (95% confidence interval [95% CI] 77\u201391%) and the specificity was 99% (95% CI 98\u2013100%).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>The 2022 American College of Rheumatology\/European Alliance of Associations for Rheumatology classification criteria for EGPA demonstrate strong performance characteristics and are validated for use in research.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/art.41982","type":"journal-article","created":{"date-parts":[[2022,2,2]],"date-time":"2022-02-02T04:01:38Z","timestamp":1643774498000},"page":"386-392","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":206,"title":["2022 American College of Rheumatology\/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis With Polyangiitis"],"prefix":"10.1002","volume":"74","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8269-9438","authenticated-orcid":false,"given":"Peter C.","family":"Grayson","sequence":"first","affiliation":[{"name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases  NIH, Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Ponte","sequence":"additional","affiliation":[{"name":"Hospital de Santa Maria, Centro Hospitalar Universit\u00e1rio Lisboa Norte Universidade de Lisboa, and Centro Acad\u00e9mico de Medicina de Lisboa  Lisbon Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ravi","family":"Suppiah","sequence":"additional","affiliation":[{"name":"Auckland District Health Board  Auckland New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7939-5978","authenticated-orcid":false,"given":"Joanna C.","family":"Robson","sequence":"additional","affiliation":[{"name":"Centre for Health and Clinical Research University of the West of England, and University Hospitals and Weston NHS Foundation Trust  Bristol UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthea","family":"Craven","sequence":"additional","affiliation":[{"name":"Oxford NIHR Biomedical Research Centre and University of Oxford  Oxford UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Judge","sequence":"additional","affiliation":[{"name":"Oxford NIHR Biomedical Research Centre and University of Oxford, Oxford, UK, and Bristol NIHR Biomedical Research Centre and University of Bristol  Bristol UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Khalid","sequence":"additional","affiliation":[{"name":"Oxford NIHR Biomedical Research Centre and University of Oxford  Oxford UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Hutchings","sequence":"additional","affiliation":[{"name":"London School of Hygiene and Tropical Medicine  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raashid A.","family":"Luqmani","sequence":"additional","affiliation":[{"name":"Oxford NIHR Biomedical Research Centre and University of Oxford  Oxford UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2846-4769","authenticated-orcid":false,"given":"Richard A.","family":"Watts","sequence":"additional","affiliation":[{"name":"Oxford NIHR Biomedical Research Centre and University of Oxford, Oxford, UK, and University of East Anglia  Norwich UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9284-7345","authenticated-orcid":false,"given":"Peter A.","family":"Merkel","sequence":"additional","affiliation":[{"name":"University of Pennsylvania  Philadelphia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"for the DCVAS Study Group","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,2,2]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.37715"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-143-9-200511010-00006"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.21250"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780330806"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10157-013-0854-0"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.21591"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.27584"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-129-5-199809010-00001"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/nri3341"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.37721"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.22003"}],"container-title":["Arthritis &amp; Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/art.41982","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/art.41982","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/art.41982","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,8]],"date-time":"2025-10-08T20:19:54Z","timestamp":1759954794000},"score":1,"resource":{"primary":{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/10.1002\/art.41982"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,2,2]]},"references-count":11,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2022,3]]}},"alternative-id":["10.1002\/art.41982"],"URL":"https:\/\/doi.org\/10.1002\/art.41982","archive":["Portico"],"relation":{},"ISSN":["2326-5191","2326-5205"],"issn-type":[{"value":"2326-5191","type":"print"},{"value":"2326-5205","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,2,2]]},"assertion":[{"value":"2020-02-28","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-09-21","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-02-02","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}