{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T15:24:46Z","timestamp":1791645886735,"version":"4.3.4"},"reference-count":156,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2021,4,1]],"date-time":"2021-04-01T00:00:00Z","timestamp":1617235200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) represents a group of small vessel vasculitides characterized by granulomatous and neutrophilic tissue inflammation, often associated with the production of antibodies that target neutrophil antigens. The two major antigens targeted by ANCAs are leukocyte proteinase 3 (PR3) and myeloperoxidase (MPO). AAV can be classified into 3 categories based on patterns of clinical involvement: namely, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA). Clinically, AAV involves many organ systems including the lungs, kidneys, skin, and nervous system. The prognosis of AAV has improved dramatically due to advances in the understanding of its pathogenesis and treatment modalities. This review will highlight some of the recent updates in our understanding of the pathogenesis, clinical manifestations, and treatment options in patients with AAV focusing on kidney involvement.<\/jats:p>","DOI":"10.3390\/jcm10071446","type":"journal-article","created":{"date-parts":[[2021,4,1]],"date-time":"2021-04-01T10:44:01Z","timestamp":1617273841000},"page":"1446","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":163,"title":["ANCA-Associated Vasculitis: An Update"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2271-500X","authenticated-orcid":false,"given":"Salem","family":"Almaani","sequence":"first","affiliation":[{"name":"Division of Nephrology, The Ohio State University Wexner Medical Center, Columbus, OH 43201, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lynn A.","family":"Fussner","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, The Ohio State University Wexner Medical Center, Columbus, OH 43201, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sergey","family":"Brodsky","sequence":"additional","affiliation":[{"name":"Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, OH 43201, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexa S.","family":"Meara","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, The Ohio State University Wexner Medical Center, Columbus, OH 43201, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Jayne","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Cambridge, Cambridg CB2 0QQ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,4,1]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1329","DOI":"10.1093\/rheumatology\/kem107","article-title":"Prevalence of Wegener\u2019s granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden","volume":"46","author":"Mohammad","year":"2007","journal-title":"Rheumatology"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"i14","DOI":"10.1093\/ndt\/gfv022","article-title":"Classification, epidemiology and clinical subgrouping of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis","volume":"30","author":"Watts","year":"2015","journal-title":"Nephrol. 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