{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T03:44:25Z","timestamp":1790567065487,"version":"4.1.0"},"reference-count":33,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2016,9,28]],"date-time":"2016-09-28T00:00:00Z","timestamp":1475020800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100000024","name":"Canadian Institutes of Health Research","doi-asserted-by":"crossref","award":["FRN TR2-119188"],"award-info":[{"award-number":["FRN TR2-119188"]}],"id":[{"id":"10.13039\/501100000024","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Pediatric Vasculitis (PedVas) Initiative"},{"DOI":"10.13039\/501100000142","name":"The Arthritis Society","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100000142","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100007211","name":"Vasculitis Foundation","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100007211","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Childhood Arthritis and Rheumatology Research Alliance"},{"name":"Child and Family Research Institute of British Columbia Children's Hospital."}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2016,10]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegener's) (GPA).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The European Medicines Agency (EMA) classification algorithm was applied by computation to categorical data from patients recruited to the ARChiVe (A Registry for Childhood Vasculitis: e\u2010entry) cohort, with the data censored to November 2015. The EMA algorithm was used to uniquely distinguish children with MPA from children with GPA, whose diagnoses had been classified according to both adult\u2010 and pediatric\u2010specific criteria. Descriptive statistics were used for comparisons.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In total, 231 of 440 patients (64% female) fulfilled the classification criteria for either MPA (n\u2009=\u200948) or GPA (n\u2009=\u2009183). The median time to diagnosis was 1.6 months in the MPA group and 2.1 months in the GPA group (ranging to 39 and 73 months, respectively). Patients with MPA were significantly younger than those with GPA (median age 11 years versus 14 years). Constitutional features were equally common between the groups. In patients with MPA compared to those with GPA, pulmonary manifestations were less frequent (44% versus 74%) and less severe (primarily, hemorrhage, requirement for supplemental oxygen, and pulmonary failure). Renal pathologic features were frequently found in both groups (75% of patients with MPA versus 83% of patients with GPA) but tended toward greater severity in those with MPA (primarily, nephrotic\u2010range proteinuria, requirement for dialysis, and end\u2010stage renal disease). Airway\/eye involvement was absent among patients with MPA, because these GPA\u2010defining features preclude a diagnosis of MPA within the EMA algorithm. Similar proportions of patients with MPA and those with GPA received combination therapy with corticosteroids plus cyclophosphamide (69% and 78%, respectively) or both drugs in combination with plasmapheresis (19% and 22%, respectively). Other treatments administered, ranging in decreasing frequency from 13% to 3%, were rituximab, methotrexate, azathioprine, and mycophenolate mofetil.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Younger age at disease onset and, perhaps, both gastrointestinal manifestations and more severe kidney disease seem to characterize the clinical profile in children with MPA compared to those with GPA. Delay in diagnosis suggests that recognition of these systemic vasculitides is suboptimal. Compared with adults, initial treatment regimens in children were comparable, but the complete reversal of female\u2010to\u2010male disease prevalence ratios is a provocative finding.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.39729","type":"journal-article","created":{"date-parts":[[2016,4,25]],"date-time":"2016-04-25T20:22:49Z","timestamp":1461615769000},"page":"2514-2526","source":"Crossref","is-referenced-by-count":137,"title":["Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study"],"prefix":"10.1002","volume":"68","author":[{"given":"David A.","family":"Cabral","sequence":"first","affiliation":[{"name":"British Columbia Children's Hospital, Vancouver  British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Debra L.","family":"Canter","sequence":"additional","affiliation":[{"name":"Texas Children's Hospital Houston"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eyal","family":"Muscal","sequence":"additional","affiliation":[{"name":"Texas Children's Hospital Houston"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kabita","family":"Nanda","sequence":"additional","affiliation":[{"name":"Seattle Children's Hospital Seattle Washington"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dawn M.","family":"Wahezi","sequence":"additional","affiliation":[{"name":"Children's Hospital at Montefiore Bronx, New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven J.","family":"Spalding","sequence":"additional","affiliation":[{"name":"Cleveland Clinic Cleveland Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marinka","family":"Twilt","sequence":"additional","affiliation":[{"name":"Alberta Children's Hospital and University of Calgary Calgary Alberta Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanne M.","family":"Benseler","sequence":"additional","affiliation":[{"name":"Alberta Children's Hospital and University of Calgary Calgary Alberta Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Campillo","sequence":"additional","affiliation":[{"name":"Montreal Children's Hospital and McGill University Health Centre Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sirirat","family":"Charuvanij","sequence":"additional","affiliation":[{"name":"Siriraj Hospital and Mahidol University Bangkok Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Dancey","sequence":"additional","affiliation":[{"name":"Janeway Children's Health and Rehabilitation Centre St. John's Newfoundland Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara A.","family":"Eberhard","sequence":"additional","affiliation":[{"name":"Cohen Children's Medical Center of New York New Hyde Park"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melissa E.","family":"Elder","sequence":"additional","affiliation":[{"name":"University of Florida Gainesville"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aimee","family":"Hersh","sequence":"additional","affiliation":[{"name":"University of Utah Salt Lake City"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gloria C.","family":"Higgins","sequence":"additional","affiliation":[{"name":"Nationwide Children's Hospital Columbus Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam M.","family":"Huber","sequence":"additional","affiliation":[{"name":"IWK Health Centre and Dalhousie University Halifax Nova Scotia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raju","family":"Khubchandani","sequence":"additional","affiliation":[{"name":"Breach Candy Hospital Mumbai India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan","family":"Kim","sequence":"additional","affiliation":[{"name":"Children's Hospital of Boston Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marisa","family":"Klein\u2010Gitelman","sequence":"additional","affiliation":[{"name":"Ann and Robert H. 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