{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,31]],"date-time":"2026-10-31T20:49:56Z","timestamp":1793479796548,"version":"4.5.0"},"reference-count":55,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2017,11,9]],"date-time":"2017-11-09T00:00:00Z","timestamp":1510185600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000049","name":"National Institute on Aging","doi-asserted-by":"publisher","award":["R01\u2010AG\u2010034676"],"award-info":[{"award-number":["R01\u2010AG\u2010034676"]}],"id":[{"id":"10.13039\/100000049","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006108","name":"National Center for Advancing Translational Sciences","doi-asserted-by":"publisher","award":["UL1\u2010TR\u2010000135"],"award-info":[{"award-number":["UL1\u2010TR\u2010000135"]}],"id":[{"id":"10.13039\/100006108","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis & Rheumatology"],"published-print":{"date-parts":[[2017,12]]},"abstract":"Objective<\/jats:title>To estimate the annual incidence, prevalence, and mortality of antineutrophil cytoplasmic autoantibody (ANCA<\/jats:styled-content>)\u2013associated vasculitis (AAV<\/jats:styled-content>) and its subsets, granulomatosis with polyangiitis (Wegener's) (GPA<\/jats:styled-content>), microscopic polyangiitis (MPA<\/jats:styled-content>), and eosinophilic granulomatosis with polyangiitis (Churg\u2010Strauss) (EGPA<\/jats:styled-content>), in a US<\/jats:styled-content>\u2010based adult population.<\/jats:p><\/jats:sec>Methods<\/jats:title>All medical records of patients with a diagnosis of, or suspicion of having, AAV<\/jats:styled-content> in Olmsted County, Minnesota from January 1, 1996 to December 31, 2015 were reviewed. AAV<\/jats:styled-content> incidence rates were age\u2010 and sex\u2010adjusted to the 2010 US<\/jats:styled-content> white population. Age\u2010 and sex\u2010adjusted prevalence of AAV<\/jats:styled-content> was calculated on January 1, 2015. Survival rates observed in the study cohort were compared with expected rates in the Minnesota population.<\/jats:p><\/jats:sec>Results<\/jats:title>Of the 58 incident cases of AAV<\/jats:styled-content> in Olmsted County during the study period, 23 (40%) were cases of GPA<\/jats:styled-content>, 28 (48%) were cases of MPA<\/jats:styled-content>, and 7 (12%) were cases of EGPA<\/jats:styled-content>. Overall, 28 (48%) of the patients with AAV<\/jats:styled-content> were women and 57 (98%) were white. The mean \u00b1 SD<\/jats:styled-content> age at diagnosis was 61.1 \u00b1 16.5 years. Thirty\u2010four patients (61%) had myeloperoxidase (MPO<\/jats:styled-content>)\u2013ANCA<\/jats:styled-content>s, and 17 (30%) were positive for proteinase 3 (PR<\/jats:styled-content>3)\u2013ANCA<\/jats:styled-content>s; 5 (9%) were ANCA<\/jats:styled-content>\u2010negative. The annual incidence of AAV<\/jats:styled-content> was 3.3 per 100,000 population (95% confidence interval [95% CI<\/jats:styled-content>] 2.4\u20134.1). The incidence rates of GPA<\/jats:styled-content>, MPA<\/jats:styled-content>, and EGPA<\/jats:styled-content> were 1.3 (95% CI<\/jats:styled-content> 0.8\u20131.8), 1.6 (95% CI<\/jats:styled-content> 1.0\u20132.2), and 0.4 (95% CI<\/jats:styled-content> 0.1\u20130.6), respectively. The overall prevalence of AAV<\/jats:styled-content> was 42.1 per 100,000 (95% CI<\/jats:styled-content> 29.6\u201354.6). The mortality rate among AAV<\/jats:styled-content> patients overall, and among patients with EGPA<\/jats:styled-content>, those with MPA<\/jats:styled-content>, and those with MPO<\/jats:styled-content>\u2010ANCA<\/jats:styled-content>s, was increased in comparison to the Minnesota general population (each P<\/jats:italic> < 0.05), whereas mortality rates among patients with GPA<\/jats:styled-content>, those with PR<\/jats:styled-content>3\u2010ANCA<\/jats:styled-content>s, and ANCA<\/jats:styled-content>\u2010negative patients did not differ from that in the general population.<\/jats:p><\/jats:sec>Conclusion<\/jats:title>The annual incidence of AAV<\/jats:styled-content> in Olmsted County, Minnesota over the 20 years of the study was 3.3 per 100,000, with a prevalence of 42.1 per 100,000, which is substantially higher than the rates reported in other areas worldwide. The incidence of GPA<\/jats:styled-content> was similar to that of MPA<\/jats:styled-content>. Patients with MPA<\/jats:styled-content> and those with EGPA<\/jats:styled-content>, but not patients with GPA<\/jats:styled-content>, experienced higher rates of mortality than that in the Minnesota general population. MPO<\/jats:styled-content>\u2010ANCA<\/jats:styled-content>s were a marker of poor survival in this population of patients with AAV<\/jats:styled-content>.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.40313","type":"journal-article","created":{"date-parts":[[2017,9,7]],"date-time":"2017-09-07T16:15:17Z","timestamp":1504800917000},"page":"2338-2350","source":"Crossref","is-referenced-by-count":204,"title":["The Epidemiology of Antineutrophil Cytoplasmic Autoantibody\u2013Associated Vasculitis in Olmsted County, Minnesota"],"prefix":"10.1002","volume":"69","author":[{"given":"Alvise","family":"Berti","sequence":"first","affiliation":[{"name":"Mayo Clinic College of Medicine and Science Rochester Minnesota"},{"name":"San Raffaele Scientific Institute Milan Italy"},{"name":"Santa Chiara Hospital Trento Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Divi","family":"Cornec","sequence":"additional","affiliation":[{"name":"Mayo Clinic College of Medicine and Science 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